A 27-year-old man suffering from incomplete type of Behçet's disease presented with swelling of the left thigh. Venography showed obstruction of the left femoral vein by thrombus, and he was diagnosed as having deep vein thrombosis of the left femoral vein. The next year, he developed recurrent hemoptysis. Pulmonary angiography showed aneurysm and mural thrombosis of the left pulmonary artery. Fiberoptic bronchoscopy showed a reddish eminence of the left Behçet bronchus, suggestive of broncho-pulmonary artery fistula. After treatment with colchicine, the hemoptysis decreased. We report a very rare case of Behçet's disease presenting with Hughes-Stovin syndrome.