Behçet disease is a systemic vasculitis characterized by recurrent oral and genital ulcers and uveitis. We describe a rare case of a 43-year-old woman with Behçet disease who was admitted for pyrexia of unknown origin, cough, dyspnea, and chest pain. Her computerized tomography scan revealed pulmonary embolism and right ventricular thrombus. She was treated with anticoagulation for pulmonary embolism and right ventricular thrombus. She was well during her last follow-up.