We report the first case of Hughes Stovin Syndrome successfully treated with long-lasting tumor necrosis factor α (TNF-α) blocker (infliximab) treatment. Because of the failure of the standard therapeutic regimen with steroids and cyclophosphamide, infliximab was started achieving a stable disease remission and a complete resolution of pulmonary aneurysms. Hughes Stovin Syndrome, although rare, is a life-threatening condition that needs to be timely identified and treated aggressively. Our report underlines the importance of TNF-α blocker treatment in Hughes Stovin Syndrome, suggesting its use as long-term safe and useful.