1. Double aortic arch can be divided into 2 types, depending upon the patency of the 2 arches. In 1 type, both arches are patent and functioning. In the other type, 1 arch, usually the left, is atretic.2. The embryologic, anatomic and angiographic features of double aortic arch with left arch atresia are discussed.3. The angiographic similarities of double aortic arch with left arch atresia to the various right arch malformations are illustrated.4. In the presence of a symptomatic vascular ring, when the angiographic findings appear to be those of a right aortic arch with mirror-image branching, the most likely diagnosis is double aortic arch with left arch atresia.