While congenital variants of the aortic arch have been well described, anatomic anomalies of the descending aorta are extremely rare. We present a case of a 31-year-old male with congenital duplication of the descending aorta resulting in advanced localized atherosclerotic disease found incidentally on diagnostic imaging. This case presents a rare anatomic variant that can not only lead to early aortic disease but may also complicate future endovascular intervention.