Abstract: Objective：To study the clinical，pathological manifestations，diagnostic and treatment methods of the ovarian nonspecific steroid cell tumors. Methods：The clinical data of patients diagnosed with ovarian nonspecific steroid cell tumors，during 2000—2010 were retrospective analyzed in our hospital. The relevant published articles were reviewed as well. Results：The two patients have been treated in our hospital during the period，one was benign and other was malignant. Both patients displayed with amenorrhea and masculine characteristics. Intraoperative and pathological findings are consistent with the descriptions of reported literatures. The benign patient was recovered uneventfully with conservative surgery and the biochemical parameters returned to normal level. The malignant one died 10 months after diagnosis with tumor recurrence despite aggressive surgery and postoperative adjuvant chemotherapy. Conclusions：Ovarian nonspecific steroid cell tumors，are rare ovarian tumor. Surgery should be preferred in treatment of such tumor. Some of the tumor was malignant and postoperative adjuvant chemotherapy should be added，while the prognosis is still very poor.

Key words: Ovarian neoplasms, Sterols, Immunohistochemistry, Diagnosis, differential, Case reports