Aneurysm formation after aortic coarctation repair is not a rare complication of post-coarctation of aorta repair. We describe the case of a 43-year-old woman who had undergone repair of an isolated interruption of the aortic arch 30 years earlier, who came to our hospital with progressive chest pain, cough and dyspnea. A giant aortic aneurysm was revealed in the distal aortic arch by CT study. The patient underwent aneurysmectomy with total aortic arch replacement using a Dacron graft through redo median sternotomy. An embryologic explanation of this patient's anomaly and the previous surgical procedure are discussed for defining this rare clinical condition.