BackgroundAzygos vein aneurysms are rare and asymptomatic in many cases. The management for these aneurysms is controversial, and there is no clear guideline or evidence-based threshold for surgical or interventional therapy.Case presentationHerein, we report the case of a giant azygos vein aneurysm in a 78-year-old man that was treated with a reversed L-shaped incision. A 56 × 77 mm saccular azygos vein aneurysm was incidentally detected on computed tomography. Subsequently, surgical resection with interventional radiology and reversed L-shaped thoracotomy was performed. First, we performed coil embolization of the azygos vein aneurysm inflow. Next, a cardiopulmonary bypass was established through a reversed L-shaped sternotomy, and the aneurysm was excised.ConclusionsIn this case, surgical resection via reversed L incision was effective.