Right-sided aortic arch with retroesophageal left subclavian artery and left ligamentum arteriosum is the second most common vascular ring. Aneurysms of the arch in its retroesophageal portion are rare. The surgical repair of a retroesophageal arch aneurysm poses a significant challenge because no single approach provides access to the whole arch and all of its branches. We describe a 39-year-old patient with aneurysmal dilatation of the retroesophageal arch who presented with airway obstruction. The arch aneurysm was repaired with a staged approach. A right-sided carotid-subclavian artery bypass was performed, followed by distal ascending aorta and aortic arch replacement under hypothermic circulatory arrest through a left thoracotomy.