Microangiopathic hemolytic anemia (MAHA) is a rare but severe complication of neoplastic disease. The diagnosis of thrombotic microangiopathy is based on a triad of a hemolytic anemia with schistocytes, thrombocytopenia, and renal failure. Carcinoma-associated MAHA and chemotherapeutic-induced MAHA have been described. Because of differences concerning prognosis and treatment it is important for the clinician to distinguish these two syndromes. However, to our knowledge, this is the first case of a sarcoma-associated thrombotic microangiopathy.