We report on a 39-year-old man with Crohn's disease who was admitted with cardiogenic shock after a short history of progressive dyspnea. Echocardiographic examination (transthoracic echocardiography) showed severe aortic regurgitation, mild mitral regurgitation, and enlargement of the sinus of Valsalva and of the ascending aorta at the level of the right pulmonary artery. The left ventricular ejection fraction was 30%. After aortic valve replacement, histologic examination of the ascending aorta showed chronic aortitis resembling syphilitic aortitis (serology for syphilis was negative) and HLA B27 related aortitis. The aortic valve showed deformation and thickening of the cusps by fibrous tissue without evidence of endocarditis. The patient remained well after surgery and echocardiographic examination 6 months later showed normal function of the aortic valve prosthesis. The diameter of the sinus of Valsalva and of the ascending aorta was slightly bigger, possibly indicating ongoing destruction. The left ventricular ejection fraction nearly normalized. It seems possible that this type of aortitis, characterized by its proximity to the valve ring, is another extraintestinal cardiac manifestation of Crohn's disease. The possibility of ongoing destruction of the sinus of Valsalva and of the ascending aorta after valve replacement makes regular echocardiographic control necessary.