Hughes and Stovin first reported a syndrome consisting of multiple pulmonary artery aneurysms and venous thrombosis in 1959. We encountered a 45-year-old man who had massive hemoptysis and leg swelling. The CT and angiography showed huge bilateral pulmonary artery aneurysms and inferior vena caval thrombosis. The bilateral lower lobe aneurysms were successfully treated with staged operations. The patient tolerated the procedures relatively well and was followed up by steroid therapy with a good response.