One case of double aortic arch with coarctation is reported. Anatomically, partial atresia of the anterior arch presented as a Kommerel's diverticulum; the coarctation was situated on the dominant posterior arch just proximal to the origin of the subclavian artery. This malformation had been diagnosed clinically during childhood. The operative indications were increasingly severe hypertension with associated left ventricular hypertrophy, and dramatic epistaxis from a nasal angioma which necessitated repeated arterial embolisation. The surgical treatment comprised the insertion of a prosthetic tube from the brachiocephalic trunk, proximal to the coarctation, to the descending thoracic aorta distal to the coarctation. The immediate operative result was excellent with return of normal peripheral pulses and blood pressure. The diagnostic difficulties of this malformation and its associations with other vascular malformations are emphasised. The surgical indications are discussed and the different techniques reviewed:--resection and end-to-end anastomosis,--tubular prosthesis,--associated procedures for double aortic arch. The choice of technique depends on the age of the patient, the anatomical conditions (double arch, coarctation, collateral circulation), and the quality of the aortic wall (atheroma).