The Sertoli-Leydig cell tumors are rare ovarian neoplasm representing only 0.1-0.5% of all primary ovarian neoplasms and are composed of sex-cord and stromal elements, that differentiated in a male direction. They are bilatere,l in less than 2% and confined to the ovaries in over 95% of cases. The majority(about two-thirds) of the reported cases are moderately or poorly differentiated Sertoli-Leydig cell tumors. We experienced one case of Sertoli-Leydig cell tumor combined with derrnoid cyst and reported this case with a review of the litenture.