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Aberrant right subclavian artery is a common congenital anomaly of the aortic arch, with a reported prevalence of approximately 0.5%. However aneurysms of this aberrant vessel are very rare.A 71-year-old man was admitted with cerebral hemorrhage. Chest X-ray revealed an abnormal upper mediastinal shadow. Angiography, computed tomography (CT) scan, and magnetic resonance (MR) imaging revealed an aberrant origin of the right subclavian artery arising as the fourth branch of the aortic arch and crossing the mediastinum from left to right indenting the esophagus posteriorly.The origin of the right subclavian artery was aneurysmal (maximum diameter was 5cm), and this aneurysm did not compress the esophagus.The patient was treated by Dacron patch graft aortoplasty and right subclavian artery reconstruction with the aid of cardiopulmonary bypass and hypothermic selective cerebral perfusion. The postoperative course was uneventful and there were no major complications.The surgical technique is detailed as well as a review of all the cases in the literature.