Hughes-Stovin syndrome is a rare disorder of unknown etiology characterized by the association of multiple pulmonary artery aneurysms and deep venous thrombosis. Some authors consider this entity an incomplete form of Behcet's disease due to the similarities between the radiologic and anatomopathological findings of pulmonary involvement. The authors report a case of Hughes-Stovin syndrome whose first venous thrombotic event preceded the development of pulmonary aneurysms by five years.