A 33-year-old man was admitted because of jaundice. Five months ago a reconstruction of the mitral valve was performed because of mitral valve insufficiency. There was a history of heroin dependence until three years ago. Laboratory tests showed normochromic normocytic anemia, increased nonconjugated serum bilirubin, decreased plasma haptoglobin, hemoglobinuria and hemosiderinuria. The peripheral blood smear contained numerous fragmented red cells, and the bone marrow showed a markedly increased erythropoiesis and absent iron stores. The clinical examination as well as echocardiography revealed severe mitral valve insufficiency; therefore, a mitral valve replacement was performed. Introperatively the mitral valve showed ruptured chordae tendineae of the posterior leaflet and a leaking stitch of the anterior leaflet. We diagnosed a macrovascular hemolytic anemia with subsequent mechanical injury and fragmentation of red cells on a dysfunctioning mitral valve.