Granulocytic sarcoma is rare extramedullary tumor composed of myeloblasts and other granulocytic precursors. The majority of cases have been reported in association with acute myeloid leukemia (AML) or myeloproliferative disorders. Granulocytic sarcoma may occur in patients with myelodysplastic syndromes. Reports are very rare, especially in the brain. We report an unusual case of granulocytic sarcoma of the parenchyma of the brain, occurring in a patient with myelodysplastic syndrome, diagnosed by cerebro-spinal fluid cytology and magnetic resonance imaging brain scan.