In this report, we have presented our experience with a patient with a rare cutaneous granulocytic sarcoma. In addition to hematoxylin and eosin, myeloperoxidase stain and specific stains for lysozyme and esterase were helpful in confirming the histologic diagnosis of granulocytic sarcoma. Despite multiple attempts to control this patient's tumor by conservative surgery, radiation therapy, and chemotherapy, we eventually had to resort to limb amputation. This procedure restored a meaningful quality of life to this patient for one and a half years prior to the development of acute leukemia. Treatment with corticosteroids at the time of surgery may have prevented a local recurrence of granulocytic sarcoma despite positive tissue margins. Our experience underscores the importance of directing treatment toward the granulocytic sarcoma whereas the myelodysplasia concurrently present may not require therapy for several years.