Cervical aortic arch (CAA) is a rare congenital anomaly. An aneurysm developed on a CAA is even rarer and a life threatening condition. We report the diagnosis and surgical treatment of an aneurysm on a CAA associated with an anomalous origin of the left main coronary artery. The surgical procedure consisted in the resection of the aneurysm, a direct aorto aortic anastomosis and a coronary artery bypass to the left anterior descending (LAD) artery with a good result at 11 months. This first case reported of an anomaly of a coronary artery origin associated with an aneurysm on a CAA, underlines the interest of a preoperative complete anatomical and functional diagnosis, to define an optimal intraoperative strategy.