Behcet's syndrome (BS) is a systemic inflammatory disease generally presented with triad of uveitis, oral and genital ulcers. However, it may present with gastrointestinal, central nervous system, skin, vascular disease manifestations. Cardiac involvement like intracardiac thrombus and valvular involvement in BS are rarely seen entities. Here we present the management of a 23-year old male BS patient who had a right ventricular thrombus and tricuspid valve dysfunction which was resistant to immunosuppressive treatment. He has been doing well for 4 years after intraventricular thrombus resection and tricuspid valve replacement with bioprosthesis.