Behçet's syndrome is a multisystem disorder presenting with recurrent oral and genital ulcerations as well as uveitis often leading to blindness. Although vascular lesions are a common complication of this disease, cardiac involvement is extremely rare. In this unusual case a young man had symptoms primarily related to recurrent right-atrial and right-ventricular thrombi unresponsive to medical treatment. The intracardiac lesion was successfully treated by surgical excision with the use of cardiopulmonary bypass. Surgical treatment should be considered in Behçet's disease complicated by cardiac thrombi, when a thrombus recurs despite medical treatment.