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Hughes-Stovin syndrome (HSS) is a systemic inflammatory condition of unknown origin that is considered to be part of the Behçet's syndrome (BS) spectrum. Recurrent venous thrombosis and superficial thrombophlebitis in combination with bilateral pulmonary artery aneurysms (PAA) represent the hallmark of HSS. The diagnostic evaluation includes computed tomography pulmonary angiography to detect signs of pulmonary vasculitis. The management of HSS is based on the European Alliance of Associations for Rheumatology (EULAR) recommendations for BS and mainly comprises immunosuppressive therapy with glucocorticoids and cyclophosphamide. In addition to drug therapy, PAA should be evaluated for interventional treatment. Spontaneous PAA rupture due to fragile vessel architecture can occur even in cases of remission and/or PAA regression., (© 2023. The Author(s).)