A 15-year-old girl with acute myelomonocytic leukemia in bone marrow remission presented with signs and symptoms of an intracranial mass lesion. A computed tomographic scan revealed a large cystic lesion in the left posterior parietal lobe, which biopsy proved to be a granulocytic sarcoma. She responded well to a combined treatment program utilizing surgical excision, radiation therapy, and intrathecal chemotherapy. Four previously reported cases are reviewed, and our findings suggest that increasing numbers of such cases may become evident as primary therapy prolongs remission duration in children with acute nonlymphocytic leukemia.