In this report we describe a case of granulocytic sarcoma (GS) developing in a patient after 66 months in complete remission of acute nonlymphoid leukemia. The granulocytic precursor cell proliferation arose simultaneously in two extramedullary sites (testis and small bowel), without evidence of bone marrow relapse. The intensive systemic chemotherapy with high-dose ARA-C allowed a clinical remission, lasting 8 months. GS eventually recurred as extramedullary multi-site disease (abdominal lymph nodes, central nervous system involvement), once again unassociated with blood and bone marrow relapse. Both, at onset and in relapse, special staining techniques on tissue sections (chloroacetate esterase) and immunohistochemical typing with monoclonal antibodies were necessary for a correct diagnosis. We discuss the relationship between systemic leukemia and the GS which showed a metastasizing tumor-like behavior.