Background: Hughes-Stovin syndrome, currently classified as a miscellaneous vasculitis, consists of one or more pulmonary artery aneurysms associated with systemic venous thrombosis. The evolution of this rare disease is commonly characterized by the rupture of the aneurysms, often responsible for a fatal fulminant hemoptysis.
Case report: Our case report refers to a young man complaining of a severe hemoptysis, who presented with a pulmonary artery aneurysm that was revealed by computed chest tomography; therefore, he underwent a right
lower lobectomy. Afew years later, an acute encephalopathy occurred, which was associated with the development of abdominal collateral blood flow and esophagogastric varices; magnetic resonance imaging showed
a thrombotic occlusion of inferior vena cava.
Conclusions: These findings suggested the diagnosis of Hughes-Stovin syndrome associated with Budd-Chiari syndrome.

Keywords: Hughes-Stovin syndrome, Budd-Chiari syndrome