A case is reported of classic granulosa cell tumor of the testis with metastases to the retroperitoneal lymph nodes occurring in a 26-year-old man. The patient had left-sided testicular enlargement and bilateral gynecomastia. He was treated by radical orchiectomy, retroperitoneal lymph node dissection, and radiation therapy and is well without evidence of disease 14 years after diagnosis. This is the first documented case of granulosa cell tumor of the testis with metastases and long remission after successful therapy to the authors' knowledge.