Development of collateral circulation belongs among the typical signs of aortic coarctation (CoA) and can lead to the growth of ectatic, fragile spinal neurovascular malformations. Spontaneous spinal epidural hemorrhage (SSEH) as a complication of CoA is exceptionally rare, with only few case reports proposed up to date. At our institute, we had two experiences of SSEH complicated by CoA. The first case had been published in 2016, with disappointing neurologic outcome. As for the second case, presenting with extremely sharp upper back pain followed by paraplegia and anal sphincter impairment, we chose immediate surgical intervention with thoracic endovascular aortic repair (TEVAR), aiming at amelioration of the pressure gradient across the juxtaductal coarctation in the aorta, to ensure the safety and the completeness of following surgical decompression of the spinal epidural hematoma. To our knowledge, this case is the first one featuring TEVAR to the CoA immediately followed by surgical decompression of SSEH in this kind of emergent setting. This case recovered satisfactorily without neurologic deficit. Conservative treatment for SSEH caused by CoA may not succeed at all times. Emergent TEVAR to CoA immediately followed by surgical decompression is achievable, making following surgical decompression of the epidural hematoma easier and safer.