Acute leukemia may be associated with coagulopathy, predominantly severe bleeding diathesis caused by disseminated intravascular coagulation (DIC) and/or hyperfibrinolysis. Disordered hemostasis is characteristic for acute promyelocytic leukemia (APL, FAB M3). However, thromboembolic events such as arterial occlusion localized to the large vessels at presentation is very rare and almost exclusively linked to APL. We report a case of severe recurrent acute arterial thromboembolism at presentation in AML FAB M1. Most likely, the ischemic events in our patient resulted from leukemia as the thrombus material included many leukemic blasts. The thrombotic complications resulted in leg amputation in this patient. Despite leg amputation just a couple of hours before and extremely high infectious risk of the patient, chemotherapy was administered. The clinical course of cessation of the ischemic events and a fast reduction of the blasts in the peripheral blood smear after chemotherapeutic treatment of the patient outlines the importance and life saving role of early chemotherapy even under adverse circumstances.