The Sertoli-Leydig cell tumor is a rare sex cord stromal tumor that has low grade malignancy and low bilateral occurrence rate. About two third of recurrence appears within 1 year after the initial treatment and most common recurrence sites are intraperitoneal cavity and retroperitoneal lymph nodes. The frequency of malignant transformation is high in the poor differentiated cell type, retiform pattern, and tumor rupture. In young women with stage I, unilateral salpingo-oophorectomy is performed to preserve uterus and reproductive function. In more advanced stages, hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymph node dissection are performed and occasionally chemotherapy can be combined. We experienced a rare case of malignant metastasis in Sertoli-Leydig cell tumor, and presented with a brief review of literature.