In this report we shall describe the first case of cervical aortic arch in which the anomaly was diagnosed clinically and the patient operated upon successfully. The lesion, an aorta with the apex of its arch located in the neck, is rare. Its importance lies (1) in the need to consider it in the differential diagnosis of pulsating neck masses; (2) in its vulnurability to trauma and susceptibility to rupture; and (3) in the need for relief of concomitant symptoms of tracheal and esophageal compression. Anatomic and clinical features of all known cases are presented with a description of noninvasive techniques for differential diagnosis. The goals of surgical therapy are division of the vascular ring with all bands and ligaments, resection of the pulsating neck mass, and restoration of aortic continuity.