Approximately 40 cases of Hughes-Stovin syndrome have been recorded in the literature. The disease process exhibits an indefinite nature. It shares histopathologic and clinical features with Behçet disease, although there is no established association. The natural course of the disease is fatal in most cases. We report a case of a 31-year-old man with Hughes-Stovin syndrome who initially presented with occasional hemoptysis and a history of deep vein thrombosis. There was evidence of right-sided lower lobe pulmonary artery aneurysm that was managed with a lobectomy, after which he developed aneurysmal recurrence in the contralateral lung. Histopathologic analysis identified myxoid degeneration in the hilar region.