Cardiac involvement is rare in Behçet's disease. Endomyocardial fibrosis of the right heart is exceptionally rare and is associated with right ventricular thrombosis. The authors report the case of a 27 year old man who died of a massive pulmonary embolism and who suffered from right-sided endomyocardial fibrosis with intraventricular thrombi. The diagnosis was made at autopsy. Behçet's disease was diagnosed on the finding of bipolar aphthous ulcers and skin hypersensitivity. The authors support previous workers in stating that Behçet's disease should be considered as a possible cause of endomyocardial fibrosis of the right heart.