Midaortic syndrome (MAS) is a rare vascular disorder characterised by segmental hypoplasia or stenosis of the thoracoabdominal aorta and possible involvement of renovisceral branches.In the case presented here, a 19-year-old female patient with thoracoabdominal aortic hypoplasia combined with multiple aortic and visceral aneurysms and a genetically confirmed COL3A1 variant (vascular Ehlers-Danlos syndrome) underwent open surgical thoracoabdominal type II aortic replacement.Using a multimodal neuro- and organ-protective approach, including perioperative CSF drainage, MEP monitoring, mechanical selective and distal extremity and organ perfusion, and targeted blood product and volume management, a complication-free thoracoabdominal reconstruction was performed.The postoperative course was normal but required subsequent interventional endovascular treatment of an anastomotic stenosis of the right renal artery.This case highlights the need for a tailored therapeutic approach for this complex, genetic aortic pathology. It demonstrates that not all aortic diseases are amenable to endovascular treatment and that open surgical treatment remains highly important, especially in younger patients with hereditary aortic diseases.