We reported a case of ankylosing spondylitis which successfully underwent aortic valve replacement for combined aortic and mitral regurgitation. A 42-year-old man was admitted with symptoms of shortness of breath and anginal pain. He was previously diagnosed ankylosing spondylitis by an orthopedician A grade III/VI to and fro murmur was audible at the left sternal border. Retrograde aortography revealed severe aortic regurgitation and mild mitral regurgitation. Cardiac catheterization showed moderately pulmonary hypertension and high pulmonary artery wedge pressure. He underwent aortic valve replacement with SJM prosthetic valve. His postoperative course was uneventful. In Japan, ankylosing spondylitis is rare disease, and cardiac lesions associated with these conditions is seldom met to us. The surgical problems and management of these lesions are discussed.