Right-sided aortic arch accompanied by an aberrant origin of the left subclavian artery is rare and seen in 0.05% approximately 0.1% of the population. A 73-year-old woman with this anomaly was admitted to our institution because of the enlargement of the distal aortic arch aneurysm. She also had mild dysphagia. The size of the aneurysm was 70 mm in diameter and she underwent total arch replacement using selective cerebral perfusion through a median sternotomy. Additional right thoracotomy was not required and four cervical vessels were reconstructed. The postoperative course was uneventful. This case report shows median sternotomy alone may provide sufficient access for this pathology.