Abstract
Retro-esophageal circumflex aortic arch is a rare form of vascular ring. Majority of cases occur in isolation without any intracardiac lesions. Only a few cases of left circumflex aorta have been reported in the literature. We present a case of left circumflex aorta with associated arch hypoplasia and coarctation, ventricular septal defect, who underwent single-stage repair.