Behçet's disease (BD) is a multisystemic, chronic, inflammatory disease of unknown origin characterized by alternation of exacerbation and quiescence phases. Cardiac involvement in BD is infrequent. We report a case of a young man with BD with right ventricular thrombus and pulmonary thromboembolism. A 20-year-old man was admitted to our hospital with a 6-month history of dyspnea at rest, asthenia, and fever. Transthoracic echocardiography showed right wall thickened and presence of floating masses in the right outflow tract. Transesophageal echocardiography confirmed the presence of diffuse thrombosis in the right ventricle, with mobile ramifications in its outflow tract. Cardiovascular magnetic resonance and computed tomography of heart confirmed the presence of thrombi, the increased thickness of endocardiac tissue, and altered cardiac wall signal transmission. Computed tomography scan showed multiple pulmonary thrombi. Myocardial biopsy specimen showed diffused subendocardial thrombosis with damage of cardiac myocytes and presence of granulocytes. Six months after discharge, no cardiac masses were detected by transthoracic echocardiography. However, a few weeks after this last echocardiogram, the patient was again hospitalized for a new episode of acute pulmonary embolism. The patient was discharged with increasing dose of oral anticoagulant, with no evidence of cardiac masses at transthoracic echocardiography and thrombosis at computed tomography. The patient is still healthy on anticoagulant, immunosuppressive, and steroid therapy. This case provides a rare example of BD, in which we found-at the same time-heart and pulmonary manifestations, with the presence of right ventricular thrombus and pulmonary thrombi in situ.