Abstract: Objective: To heighten the apprehension of the clear cell carcinoma of ovary with hyperandrogenism and to avoid misdiagnosis. Methods: A rare case of primary clear cell carcinoma of ovary with amenorrhea and virilization was reported. Through reviewing the literature and discussing the mechanisms of hormone production by these tumors we want to knowhowto make differential diagnosis with other tumors. Results: A primary clear cell carcinoma of ovary was revealed by Histology. The abnormal hormone value was normalized two weeks after operation. But the case show an aggressive course and did not respond to chemotherapy. The patient died of the disease 6 months after the operation. Conclusion: Ovarian neoplasm, other than sex cord stromal tumor, is the rare cause of hyperandrogenism. Only three cases of primary clear cell carcinoma of ovary is associated with virilization have been reported. Differential diagnosis should be done with sex cord stromal derivation tumor, steroid cell tumor, yolk sac tumor and metastatic tumor. The main basis for diagnosis is histology. Immunohistochemistry may be helpful in differential diagnosis.