In Behcet syndrome, cardiac involvements are rare and have been reported pericarditis, myocarditis, right heart endocardial fibrosis, right ventricle mural thrombus with pulmonary embolism, active endocarditis, granulomatous endocarditis, conduction disturbance, acute aortic insufficiency, mitral valve prolapse. Our three patients underwent AVR because of aortic insufficiency and ascending aorta enlargement combined with Behcet syndrome. Two patients had mitral regurgitation too. So one underwent MAP and the other underwent MVR concomitantly. One who underwent AVR have been well for 50 months. Another who underwent AVR+MAP and redo AVR due to aortic paravalvular leakage was died of congestive heart failure. The other who underwent AVR+MVR and repeated AVR three times because of aortic paravalvular leakage is in condition of aortic paravalvular leakage. Paravalvular leakage is considered to recur due to progressive dilatation and fragility of aortic root that is the result of pathologic change of Behcet syndrome in it. If Open heart surgery is needed in Behcet`s syndrome during inflammatory reaction is active, postoperative complications such as paravalvular leakage or suture line rupture may be prevented with pre- and postoperative anti-inflammatory management.