We herein describe our These authors contributed equally to this work.experience with a congenital innominate artery aneurysm (IAA) that was managed with a simple surgical procedure. A 44-year-old woman was admitted for chest distress. Computed tomography angiography showed a 3.6-cm IAA arising from the aortic arch and compressing the trachea. A median sternotomy was performed with the patient under general anesthesia, and the IAA was found to involve the origin of the innominate artery and the bifurcation of the right subclavian artery and common carotid artery; however, the aorta was intact. An 8-mm Dacron graft was anastomosed to the ascending aorta and distal end of the IAA without cardiopulmonary bypass. The postoperative course was uneventful, and repeat computed tomography angiography revealed no evidence of recurrence 6 months postoperatively. We also herein present a literature review of this rare clinical condition.