The incidence of neurovascular complications with paraplegia in patients of coarctation of the aorta (CoA) is very rare, and the prognosis of the outcome is poor. The traditional standardized management for the aortic coarctation is surgical repair, which can be associated with considerable intraoperative risk and postoperative morbidity. In this article, we present a case of diagnosis of the primary CoA complicated with acute epidural hematoma and paraplegia treated with endovascular repair with balloon angioplasty and stent placement successfully.