A 67-year-old woman with rheumatic aortic stenosis for 20 years was admitted to our hospital. Although she had no overt symptoms, she had severe aortic valve stenosis with a transvalvular pressure gradient of more than 150 mmHg. She had also been suffering from anemia and mild chronic renal failure. A peripheral blood smear showed numerous fragmented erythrocytes. Hemoglobin was 8.4 g/dl, lactate dehydrogenase was 316 IU/l, haptoglobin was less than 7.3 mg/dl, and hemosiderinuria was evident. We diagnosed intravascular hemolysis related to aortic stenosis. After we performed an aortic valve replacement, fragmentation on the peripheral blood smear dramatically disappeared.