An aberrant right subclavian artery from the descending aorta is almost always reported as an isolated anomaly. We present the case of a four-year-old child with an anomalous origin of the right subclavian artery from the descending aorta, associated with an ostium secundum atrial septal defect. The patient underwent simultaneous repair of both the anomalies through median sternotomy, with implantation of the subclavian artery into the right common carotid artery. We believe that median sternotomy is the optimal surgical approach for the management of these lesions. Other operative approaches are also discussed.