A 26-year-old woman with intermittent fever was admitted to our hospital, and gradually developed facial edema. Examinations including computed tomography, transesophageal echocardiography, digital subtraction angiography, and pulmonary perfusion scintigraphy revealed intracardiac thrombus, superior vena cava syndrome, and pulmonary embolism. Clinical findings and laboratory data led us to make a diagnosis of Behçet's disease. Combination of intracardiac thrombus, superior vena cava syndrome, and pulmonary embolism are rare complications in Behçet's disease. Behçet's disease should be considered in the differential diagnosis of intracardiac mass of the right heart, and early diagnosis and treatment are essential for the management of Behçet's disease especially with large-vessel manifestations. In addition to a case report, we review the literature and report the characteristics of intracardiac thrombus in Behçet's disease.