A young man presented with intestinal obstruction. Laparotomy revealed a large mass encircling the cecum and terminal ileum which histologically proved to be a granulocytic sarcoma. No evidence of leukemia was present in peripheral blood or bone marrow. Initial therapy consisted of abdominal irradiation. Two months following completion of irradiation, widespread systemic involvement without leukemia was evident. Systemic chemotherapy with doxorubicin, cytosine arabinoside, vincristine, and prednisone was initiated. Chemotherapy was continued for 18 months. The patient has remained without evidence of disease for 49 months following discontinuation of treatment, and 67 months after initial diagnosis. Systemic therapy for granulocytic sarcoma presenting without evidence of leukemia is reviewed.