Both perivalvular leakage and kinked prosthetic graft may cause hemolysis. A 72-year-old man was refereed to our hospital because of hemolytic anemia. He has past histories of total aortic arch replacement and repeat aortic valve replacement for aortic aneurysm and prosthetic valve endocarditis. Pre-operative examinations demonstrated aortic valve perivalvular leakage and severe graft kinking of the elephant trunk. Repeat aortic valve replacement and axillo-femoral bypass were performed successfully. Hemolysis got better after the operation and the patient discharged home in stable condition.