A 14-year female was admitted to the hospital with fever for seven days and jaundice for four days. Seven days before admission, the child developed fever, the maximum body temperature was 38°C, accompanied by abdominal pain, diarrhea, take one tablet of compound paracetamol (each containing acetaminophen 250 mg, Amantadine hydrochloride 100 mg). Four days ago, she had abdominal distension, jaundice. The laboratory data were as follows: the 1st day of admission: activated partial thromboplastin time (APTT) 67.4 s, prothrombin time (PT) 36.5 s, INR 3.46, prothrombin activity (PTA) 20 %, fibrinogen (FBG) 1.30g/l; aspartate aminotransferase (AST) 161.5 U/L (normal range: 15-40U/L), alanine aminotransferase (ALT) 15.3 U/L (normal range: 9-50U/L), γ-glutamyltransferase (GGT) 135 U/L, total bilirubin (TBIL) 1013.5 umol/L (normal range: 0-26 μmol/L), direct bilirubin (DBIL) 645.5 umol/L (normal range: 0-6.8 μmol/L), indirect bilirubin (IDBIL) 368.0 umol/L (normal range: 5-20 μmol/L);white blood cell 25.05×109/L, neutrophil percentage 0.78, lymphocyte percentage 0.11, red blood cell 1.97×1012/L, hemoglobin 74 g/ L, platelet 209×109/L;C-reactive protein（CRP） 12.64 mg/L; procalcitonin 9.15 ng/mL; Ascites routine: protein 5.48 g/L, white blood cells 114×106/L, red blood cells 100×106/L, mononuclear 0.61, troponin 1.29 ng/ml; BNP 2660 pg/ml. The 2nd day of admission: white blood cells 28.06×109/L, lymphocyte percentage 0.13, red blood cells 1.47×1012/L, hemoglobin 59 g/L, platelets 163×109/L; AST 210.0 U/L, ALT 17.4 U/L, γ-GGT 118.1 U/L, TBIL 1302.8 umol/L, DBIL 779.2 umol/L, IDBIL 523.6 umol/L; APTT 66.8 s, PT 34.1 s, INR 3.34, PTA 20%, FBG 0.83g /l; NSE 88.80 ng/ml, ceruloplasmin 0.121 g/L (0.2-0.5 ); lactate dehydrogenase (ascites) 58 U/L; adenosine deaminase (ascites) 1.1 U/L; ferritin 5610.3 ug/L, complement C3 0.58 g/L, complement C4 0.06 g/L; urine bilirubin 3+, urine occult blood 3 +, urine protein 2 +, urine Copper 30.18 umol/24 h; blood ammonia 80 umol/L; hemolysis test, erythrocyte sedimentation rate, hepatitis A, hepatitis B, hepatitis C, hepatitis D, hepatitis E, blood lipids, amylase, lipase, thyroid function, EB,CMV antibody and nucleic acid quantification, respiratory pathogens, ANA series, autoantibody detection and blood culture were normal. Abdominal color Doppler ultrasound: the liver parenchyma had fine and enhanced echos, the portal vein blood flow direction was away from the liver, cholestasis in the gallbladder, fluid in the abdominal cavity, ascite. Lung CT: inflammation of the lungs, pleural effusion. Head MRI and CT findings were normal. Enhanced CT of Abdomen: large patches of liquid density shadows around the liver and spleen, abdominal cavity, pelvic cavity, and gastric varicose veins. Liver pathology: nodular liver cirrhosis, focal submass parenchymal necrosis, according to severe hepatitis, it is recommended to combine genetic testing to exclude liver metabolic diseases. Gene detection: Sanger sequencing of ATP7B gene identifified a homozygous nonsense mutation c.3955C>T (p.Arg1319Ter) in exon 13. When the child was admitted to the hospital, she was conscious, had vomiting, abdominal distension, and could communicate. We supplement prothrombin complex, vitamin K1, fibrinogen, plasma infusion to improve coagulation, reduced glutathione, hepatocyte growth-promoting hormone, and Simetech for treatment. On the 2nd day of admission, the patient became unconsciousness, irritability, with a Glasgow Coma Scale (GCS) score of 6. Hemoglobin decreased, liver function and coagulation routine continued to deteriorate. Acute liver failure and hepatic encephalopathy were present, so she was transferred to the pediatric intensive care unit for furture treatment, plasma exchange combined with dual plasma adsorption (DPMAS), blood purification were used. We evaluate the changes in the nervous system daily, calculate the liver injury unit score, and evaluate the timing of liver transplantation. Meanwhile, we find the cause of liver failure: the child had a history of fever, abdominal pain, and diarrhea, we should pay attention to enterovirus infection; the child is an adolescent girl, she did not have joint pains, skin rash, light allergy, joint pain, hair loss. ANA series were normal; She had oral acetaminophen drugs within the normal drug dose. The parents denied the history of toxicosis, so impossibility of poisoning and immune factors; the child had acute liver failure and hemoglobin decrease, considered the existence of non-immune hemolytic anemia, ceruloplasmin 129 mg/l (200-500mg/l), lower than normal, 24 h urinary urinary: 30.18 umol (1886 ug) (normal<100 ug), should be alert Hepatolenticular degeneration, so we send the hepatolenticular gene for furture examination which Got from parents. After six days of treatment, coagulation routine and bilirubin improved, and she was gradually conscious. She can communicate with people normally, express emotions. On the 8th day of admission, she developed dyspnea, and had bloody ascites, bleeding around the drainage tube and deep venous catheterization, and oral mucosa was bleeding. The abdominal color Doppler ultrasound examination showed that the portal vein blood flow direction was away from the liver. Abdominal CT showed gastric varicose veins. The condition continued to deteriorate, she was confusion, and had dyspnea, bleeding. The coagulation routine continued to deteriorate, bilirubin did not decrease significantly. Liver transplantation was considered. Intraoperative liver pictures was shown in Figure 1. Postoperative liver pathology suggested nodular cirrhosis. The gene identified: ATP7B gene mutation is heterozygous mutation, according to the diagnosis of hepatolenticular degeneration. After the operation, she was given anti-infection, glucocorticoid, tacrolimus, mycophenolate mofetil anti-rejection therapy, anticoagulation, prevention of arterial thrombosis, respiratory support. After the operation, her condition improved, she was conscious and the ventilator was removed. The blood flow of the portal vein was normal, no venous thrombi, and no other systemic complications occurred, liver function gradually returned to normal, head MRI were normal. Thity-three days later, she recovered.