INTRODUCTION:Intrapulmonary sequestration (ILS) is a rare congenital entity characterized by a nonfunctional lung tissue supplied by an aberrant systemic artery.Given the inherent risks of intraoperative haemorrhage resulting from failure to control these vessels, a limited but growing number of cases have been treated by hybrid surgical approach.We present the case of a young adult with symptomatic ILS who was successfully treated with staged, thoracic endovascular aortic repair (TEVAR) and left lower lobectomy.PRESENTATION:A 24-years old patient, with a medical history of chronic recurrent lower respiratory tract infections, was presented with high fever  and sever increase in serum inflammation markers.The CTA scan revealed a complicated ILS supplied by 4 aberrant vessels.A hybrid, three-staged treatment was proposed.Patient firstly received an i.v 10 days meronen/linezolid antibiotic scheme and negative blood culture tests were confirmed before hospital discharge. Sequentially, TEVAR was implanted with the use of a short stent graft inserted by the left femoral access and deployed with a 2 cm proximal and distal landing zone to the origins of the aberrant arteries. A post-TEVAR completion angiogram confirmed coverage of all feeding arteries, with no ongoing antegrade filling. Six days later, an uneventful left lower lobectomy was performed, and the patient was discharged on the 3rd post-op day.CONCLUSION:To our knowledge this is the first published case report of an ILS receiving direct supply by four aberrant arteries. It supports emerging cases demonstrating accumulating benefits and improved outcomes of endoluminal aortic stenting followed by surgery in selected ILS patient population.