Introduction: Pulmonary sequestration is a rare congenital lung malformation characterized by non-functioning lung tissue that lacks communication with the tracheobronchial tree and derives its blood supply from the systemic circulation. It commonly presents with recurrent or non-resolving pneumonia, particularly in early childhood. Case presentation: A 1-year and 7-month-old girl, the second child of consanguineous parents, born at term with no perinatal complications, presented with recurrent episodes of pneumonia. At 1 year and 4 months of age, she was first admitted with fever, cough, and respiratory distress. Examination revealed reduced air entry over the left middle and lower lung zones. Laboratory investigations showed leukocytosis (30.4 × 10? /L), thrombocytosis (904 × 10? /L),and elevated Creactive protein (178 mg/L). Chest radiography demonstrated opacification in the left middle and lower zones, and she was treated as left lower lobe pneumonia with broad-spectrum intravenous antibiotics. Although initial clinical improvement was noted, she required two further admissions within one month for recurrent pneumonia involving the same region, with persistent radiographic changes. Contrast-enhanced CT with CT angiography confirmed intralobar pulmonary sequestration supplied by an aberrant artery arising from the descending thoracic aorta (T9–T10) with pulmonary venous drainage. Surgical management via thoracoscopy , converted to thoracotomy due to dense adhesion, included ligation of the feeding artery and left lobectomy . The child recovered uneventfully . Conclusion: Pulmonary sequestration should be considered in children with recurrent, localized pneumonia unresponsive to appropriate medical therapy .