An unusual case of a 44-year-old patient with primary left testicular granulocytic sarcoma is presented. Iliac and paraaortic lymph node involvement was also demonstrated at staging laparotomy. The patient was treated with left orchiectomy and early intensive chemotherapy, but relapsed with a right testicular mass 14 months later. No evidence of leukemic progression was found at that time, and he was treated with right orchiectomy, combined with irradiation to the scrotum, inguinal, iliac and paraaortic lymph nodes. Unfortunately, he developed myelogenous leukemia 7 months later. A complete remission of 6 months duration was achieved with combination chemotherapy. The patient then relapsed, and an effort to induce a second remission was unsuccessful. He eventually died almost 3 years after initial diagnosis. We conclude that primary granulocytic sarcoma represents a systemic disease. Despite early intensive treatment its prognosis remains poor.