Objective To analyze the clinical characteristics, diagnosis, and treatment of ovarian juvenile granulosa cell tumor.

Methods The clinical and pathological data of six patients with ovarian juvenile granulosa cell tumor was collected.

Results The mean age of disease onset was 20.5 years (range 12 to 33). All six patients had an adnexal mass located laterally in the pelvis, and two developed ascites. All patients had fertility-sparing surgery with complete staging. The mean size of the tumors was 15.3 cm (range 5 to 35). Ovarian sex cord stromal tumors were diagnosed or highly suspected from the frozen sections for all patients. Five patients received three to six courses of postoperative adjuvant chemotherapy, with three receiving a bleomycin/etoposide/cisplatin regimen and two receiving a paclitaxel/carboplatin regimen. The five stage I patients had no recurrence with 52 to 155 months of follow-up. The patient with stage IIIB disease had a recurrence 55 months’ later and underwent reoperation and chemotherapy. This patient remained disease-free 30 months after the reoperation.

Conclusions Fertility-sparing surgery is the treatment of choice for ovarian juvenile granulosa cell tumor and the overall prognosis is good.