Behçet's disease (BD) is a chronic inflammatory multisystem disorder characterised by recurrent oral and genital aphtosis and ocular involvement. Thrombophlebitis and major vessel thrombosis are common manifestations of vascular involvement in BD patients, whereas intracardiac thrombosis is extremely rare. We describe a 22-year-old woman who presented with deep vein thrombosis and recurrent right ventricular thrombosis complicated by pulmonary embolism. At the time, she complained of fever, recurrent painful oral and genital aphtae and papulo-pustular skin rash so she was diagnosed with BD. She received intravenous streptokinase 50,000 units/hour for three days plus corticosteroids with complete recovery. A review of intracardiac thrombosis in BD is presented and the use of thrombolytic therapy in this rare condition is briefly discussed.