Malignant Leydig cell tumors (LCT) are rare. Only 32 cases of malignant LCT (as evidenced by metastatic spread) were reported. Generally metastatic spread occurs within 2 years of the primary LCT, and the patient dies within 2 years of the discovery of metastatic disease. The tumor is highly resistant to both radiation and chemotherapy. It also has a great propensity for recurring after surgical resection. A case is reported of a patient whose metastatic disease occurred 8 years after his primary LCT had been resected. He was treated with doxorubicin and mitotane without response. The clinical features of this case are highlighted, and a review of the literature describing treatment of this rare disease is presented. Cancer 68:2324–2329, 1991.