A retroesophageal aberrant brachiocephalic artery is a very rare congenital aortic arch anomaly. We herein presented a 29-year-old man with right aortic arch, retroesophageal aberrant left brachiocephalic artery, left ligamentum arteriosus, and absent left internal carotid artery. Graft replacement of the descending aorta and anatomical reconstruction of left brachiocephalic artery was successfully performed using a midline sternotomy approach without blood transfusion. We discuss the surgical management for Kommerell's diverticulum.
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