Aberrant right subclavian artery is a rare condition with a prevalence of 0.5%-2.0% of the population. We report a case of distal aortic arch aneurysm with right subclavian artery. A 75-year-old man who was asymptomatic was referred to our hospital for a thoracic aortic aneurysm. Computed tomography showed a 55-mm fusiform aneurysm of the distal arch and an aberrant right subclavian artery. Total arch replacement was performed via median sternotomy with antegrade selective cerebral perfusion and hypothermic circulatory arrest. We reconstructed the aberrant right subclavian artery in the normal position to avoid compression of the esophagus and trachea caused by future aneurysmal dilatation of the orifice of the aberrant right subclavian artery and potential high risk for rupture.