A case of intralobar pulmonary sequestration presenting with recurrent episodes of infection is reported. Pulmonary sequestration is a rare congenital malformation characterized by accessory lung tissue with no direct connection to the tracheobronchial tree and is supplied by an aberrant systemic artery. The aberrant artery is key to both diagnosis and treatment. The correct diagnosis may be suspected on clinical grounds and should be confirmed by identifying the lesion and aberrant artery on imaging studies. Surgical resection is curative, taking particular care not to injure the aberrant artery, which may result in devastating intraoperative hemorrhage.