46-year-old female patient was undergoing the third recurrence after primary treatment for adult GCTs. Total abdominal hysterectomy, bilateral salpingo-oophorectomy with pelvic and abdominal para-aortic lymph node dissection was performed under open surgery for the Ia stage of left ovarian granulosa cell tumor in 2005. During the 10 years of postoperative regular follow-up. the tumor marker(including CA12-5, CA19-9, CEA, CA153, AFP, AMH, inhitbinA) were normal as well as imaging evaluation .In February 2017, with the complaint of lower abdominal pain with abdominal distension, no nausea, vomiting, bloody stool and other discomfort, she was referred to the Gynecology of the Fifth Affiliated Hospital of Sun Yat-sen University. A mass of 73 x62mm was found during pelvic midline region by total abdominal MRI scan. Local recurrence of granulosa cell tumor was Considered, and laparoscopic pelvic mass resection was performed. Intraoperative explored reveal A mass of about 8.0 x6.0cm was found in the middle pelvic cavity with unclear boundary, Adhension to the bowel, small intestine and sigmoid distorts, Part of the intestinal serous layer is invaded by tumors, the surgeons completely remove the mass without excised intestines. Postoperative pathology confirmed the recurrence of ovarian granulosa cell tumor. Immunohistochemistry showed tumor cell :Vimentin(+), CD99(+), inhibi(+).However, postoperative chemotherapy was refused. In May 2018,CT scan showed : Multiple masses Located in retroperitoneum, liver and kidney recess(Figure 1A), peritoneum and pelvic cavity(Figure 1B,1C) .Considering metastatic tumor with partial bleeding, No significant changes in pelvic and abdominal tumors were assessed after treatment with Combined paclitaxel 240 mg and cisplatin 100mg for 6 cycles, The level of Serum creatinine Elevated, was diagnosed with drug - induced interstitial nephritis, symptomatic treatment was given. in July 2019,MRI scan found: multiple metastatic tumors of liver and kidney recess, pelvic wall peritoneum and pelvic cavity with partial hemorrhage, The lesion was slightly enlarged(Figure 1D), the third Optimal cytoreduction to no residual disease was performed on 16 July 2019, Intraoperative exploration reveal 2.0 x3.0x2.0cm Metastatic neoplasm located in the right pelvic cavity, 1.5 x2.0x3.0cm Metastatic neoplasm in the left pelvic cavity,about 8.0x7.0x7.0 cm Metastatic neoplasm transposited the anterior wall of the sigmoid rectum, encapsulated by the gut, Infiltrated growth, Multiple localized tumors located in the peritoneum, A localized tumor mass of about 5.0x5.0x4.0 cm, was seen in the peritoneum of the hepatic and renal recess, no enlarged lymph nodes was found, No significant tumor was found on the surface of liver and diaphragm, the surgeons completely removed all visible metastatic tumors with partial sigmoidectomy and intestinal anastomosis ,Postoperative pathologic findings: Metastatic ovarian granulosa cell tumor, D99(+), CD56(+), Ki67(10%+)(Figure 1D), Postoperative adjuvant treated with the regimens of Letrozole 2.5mg qd ,A total abdominal CT scan was reviewed in November 2019,No abnormality was found(Figure 2A), Continued to be treated with letrozole. But in February 2020 ,The MRI scan showed a 3.0 x2.5cm Metastatic neoplasm located abdominal para-aorta(Figure 2B), Letrozole resistance was diagnosed,after MDT consultation,we try to Experimental treatment with Diphereline 3.75mg im q28d for 3 cycles, the size of Metastatic neoplasm Reducted to 1.3 x0.5cm under the CT scan in August 2020(Figure 2C), Continued to be treated with Diphereline ,In February 2021, CT scan showed the Metastatic neoplasm disappear (Figure 2D),achieved clinical cure, So far, PFS reached 12 months, Proposed continued the current programme treatment