The recognition of a double aortic arch which causes significant constriction of the esophagus and trachea is relatively easy if the cardiac symptoms caused by this anomaly are borne in mind. Once suspected, the diagnosis may be confirmed readily by radiographic examination of the esophagus with a barium swallow. The treatment consists of division of the ductus arteriosus (or ligamentum) and of the smaller component, usually the anterior, of the double aortic arch.