Background: This report describes one case of anesthetic management about surgical resection of a malignant phaeochromocytoma with tumor extension into vena cava and right atrium in a patient. Report for anesthetic management is limited in these patients under surgical resection until now. Case presentation: In September 2015, a 24-year-old male presented to the department of cardiology with right flank pain and hypertensive urgency in our hospital. Contrast-enhanced CT abdomen and MRI abdomen revealed a mass phaeochromocytoma in right adrenal, which invaded the right inferior vena cava（IVC）wall along with IVC thrombus. Echocardiography shown no abnormal detection. Finally, this patient gave up the surgical resection of phaeochromocytoma and chose the expectant treatment. In April 2018, this patient once again presented to the emergence department in our hospital, he had experienced persistent cough and intermittent wheezing for 5 hour. Contrast-enhanced CT and echocardiography shown existing IVC thrombus had extended into the right atrium. After the careful preoperative preparation, adrenalectomy with complete thrombus excision by inferior vena cava exploration and right atriotomy were performed successfully by a multidisciplinary team. After one month post-operation care, this patient healthily left our hospital. Conclusion: To the best of our knowledge, the occurrence of pheochromocytoma with IVC and right atrium thrombosis has not been reported in mainland China so far. This clinical case may supply a rare reference experience for surgical treatment and anesthetic management in the group of phaeochromocytoma patient with distance vascular extension.