The diagnosis of a case of childhood orbital granulocytic sarcoma, initially classified as a poorly-differentiated malignant tumor, was finally considered at the time of relapse following primary treatment with chemotherapy and local irradiation. At this time, systematic cerebrospinal fluid examination showed numerous blast cells with Auer rods, consistent with the diagnosis of meningeal acute myeloid leukemia of M2 type, and concomitant biopsy of the tumor indicated the diagnosis of chloroma. Reevaluation of the primary biopsy confirmed this diagnosis.