Background <p>Bronchopulmonary sequestration (BPS) is a rare congenital pulmonary anomaly characterized by nonfunctional lung tissue that lacks communication with the tracheobronchial tree and receives systemic arterial supply. Although BPS is typically localized and unilateral, its coexistence with end-stage bilateral bronchiectasis is exceedingly uncommon and presents major diagnostic and surgical challenges.</p> Case Presentation <p>We report a 15-year-old Syrian female with recurrent lower respiratory tract infections since infancy and chronic daily productive cough. Chest CT demonstrated extensive bilateral lower-lobe bronchiectasis. Contrast-enhanced CT angiography identified an aberrant systemic artery arising from the descending thoracic aorta supplying the left lower lobe, consistent with intralobar sequestration (ILS). A staged open surgical strategy was adopted. The first stage comprised right lower lobectomy with wedge resection of the right middle lobe and bronchial stump buttressing using a pedicled fifth intercostal muscle flap. One month later, the second stage consisted of left lower lobectomy with intraoperative ligation and division of the systemic feeding artery. Histopathology confirmed severe bronchiectasis with abscess formation, hemorrhage, and vascular congestion, without malignancy or fibrosis. At 18-month follow-up, the patient had complete resolution of productive cough and no evidence of bronchopleural fistula.</p> Conclusion <p>This case illustrates an exceptionally rare coexistence of unilateral ILS with end-stage bilateral bronchiectasis. In the setting of extensive inflammatory destruction, staged open resection with proactive bronchial stump reinforcement can be a safe and effective strategy. Early CT angiography and multidisciplinary assessment should be considered in children and adolescents with recurrent infections and progressive bronchiectasis to avoid delayed diagnosis and guide individualized surgical planning.</p>