We report the case of a 13-year-old girl with a diffusely hypoplastic right aortic arch, anomalous origin of the left subclavian artery, and a small, insignificant ventricular septal defect. The patient's pulse was forceful at the carotid arteries, but it was markedly weaker at all extremities. Catheterization revealed that both common carotid arteries arose from the dilated ascending aorta; the right subclavian and vertebral arteries arose from the hypoplastic posterior segment of the aortic arch, and the left subclavian artery arose from the normally developed descending aorta. The pressure gradient between the ascending and descending aorta was 80 mmHg. A bypass grafting procedure was performed to connect the ascending and the upper abdominal aorta. No pressure gradient remained after the operation, nor was a gradient detected during 2 years of follow-up. The origin of both subclavian arteries distal to the area of coarctation resulted in an atypical clinical picture and delayed diagnosis. Ten previously reported cases of coarctation of the aorta with right aortic arch are reviewed.