A 34-year-old man underwent left hemicastration for malignant unclassified sex cord gonadal stromal tumor. At 6 months pulmonary metastases developed and the patient received 3 courses of chemotherapy consisting of cisplatin, bleomycin and etoposide. A residual focus in the right lung was excised and proved to be viable tumor. He then received 2 adjuvant courses of cisplatin, etoposide and ifosfamide. Six months later he was without evidence of disease. A review of the literature revealed 21 previous cases of malignant unclassified sex cord gonadal stromal tumor. Although chemotherapy usually fails in treating Leydig cell tumors our case corroborates 6 previous reports of favorable response to cisplatin-based chemotherapy. This finding suggests that different subtypes of sex cord gonadal stromal tumor respond differently to chemotherapy.