Background <p>Coarctation of the aorta represents 5% of all congenital cardiac anomalies, with isolated coarctations occurring in 3 per 100,000 live births. Repair in adulthood include open, hybrid, and endovascular options – with prosthetic interposition tube grafts being the preferred surgical technique for adult patients wherein a graft is sewn into the anatomical pathway after resection of the coarcted segment. Described complications of this technique include re-coarctation or aneurysm development distal to the tube graft. We herein report the feasibility of total arch frozen elephant trunk (FET) repair and subsequent thoracic endovascular aortic repair (TEVAR) for a thoracic aortic aneurysm after previous extra-anatomic repair of coarctation.</p> Case presentation <p>A 72-year-old female with poorly controlled hypertension was found to have a distal arch coarctation for which she underwent repair with an extra-anatomic prosthetic tube graft. Post-operative surveillance revealed the development of post-stenotic dilatation of the proximal descending thoracic aorta (5.9 cm). For this the patient underwent a staged repair, first with a total arch FET replacement using an integrated 26x150mm Thoraflex Hybrid Ante-Flo device. Followed by a TEVAR with a 26x195mm Relay Pro device.</p> Conclusions <p>To the best of our knowledge this is the first reported case of a Thoraflex Hybrid graft being implanted through an extra-anatomic interposition graft with a second staged TEVAR for aneurysm isolation. This unique case exemplified the need to have complex aortic cases done at a comprehensive aortic center where cases like these are tackled by a multi-disciplinary team.</p>