Cardiac valve disease resulting from elastic tissue defects has been demonstrated in patients with beta-thalassemia; however, valve disorders of patients with alpha-thalassemia have been rarely discussed. We present the case of a patient with alpha-thalassemia and severe aortic regurgitation with left ventricular dysfunction. The patient underwent successful aortic valve replacement with mechanical prosthesis. Histopathology of the excised valve showed elastic tissue disruption and chronic thrombus on the ventricular side. Hypercoagulative states have been observed in patients with alpha-thalassemia as beta-thalassemia; therefore special attention should be taken in perioperative anticoagulation therapy.