Metastatic Sertoli cell tumor is a very rare and deadly disease accounting for approximately 1% of all testicular carcinomas. With fewer than 30 cases reported in the literature, there has not been a uniform treatment regimen with good results. Retroperitoneal lymph node dissection, chemotherapy, and radiation therapy in combination appear to offer the best outcome. This report describes the occurrence of this rare tumor in a 38-year-old man 2 years after left orchiectomy.