Although an aberrant right subclavian artery arising from the proximal portion of the descending thoracic aorta is the most common aortic arch anomaly, few patients have clinical symptoms directly attributable to it. When symptoms do occur they are usually causally related to aneurysmal or occlusive sequelae of atherosclerotic disease of the anomalous vessel. More unusual manifestations peculiar to the anomalous artery include aneurysmal degeneration of the origin of the vessel from the aortic arch, with its inherent risk of rupture, or symptoms of compression of the trachea or more commonly the esophagus by the anomalous vessel as it traverses the superior mediastinum. In patients with symptoms a variety of operative approaches and management strategies have been used. Our recent experience with treatment of two patients with clinical symptoms caused by an aberrant right subclavian artery illustrate the varied surgical options and prompted a review of the surgical management of this unusual anomaly.