We report a rare case of a 39-year-old man with type A aortic dissection involving a right-sided aortic arch with the symptom of vascular ring. Computed tomography scanning and angiography were performed to define the extent of the dissection and the anatomy of the branching vessels. The ascending aorta was replaced through a median sternotomy and right thoracotomy using a hypothermic cardiopulmonary bypass associated with selective cerebral perfusion and partial circulatory arrest, and his symptom of vascular ring disappeared postoperatively.