A right aortic arch with a retroesophageal brachiocephalic artery is a very rare type of vascular ring. We present a case report along with a review of published cases to date. Twelve publications from 1968-2014 describe a retroesophageal brachiocephalic artery in a total of 13 patients. The mean age at diagnosis was 8.7 ± 16 years. Four of the 13 patients were boys. Nearly half of the patients were symptomatic, with dysphagia or respiratory difficulties. Ten patients (77%) had associated congenital heart defects. Of the 13 patients with retroesophageal brachiocephalic artery, 12 had a right aortic arch, and only 1 had a left aortic arch associated with complex congenital heart disease.