We performed two-stage repair for a rare adult case of interrupted aortic arch with aortic regurgitation and sinus of Valsalva aneurysm. A lateroisthmic bypass was established with minimal thoracotomy and partial clamping of the descending aorta to preserve collateral circulation. This was followed by aortic root reconstruction with a prosthetic graft and valve for aortic regurgitation with sinus of Valsalva aneurysm. This less invasive two-stage repair for such a rare pathology may facilitate smooth recovery of the patient.