Bronchopulmonary sequestration represents a spectrum of abnormalities. One of these abnormalities is an aberrant systemic arterial supply to a normal lung with no bronchial sequestration. These lesions were originally classified by Pryce as type 1. Most of these patients are asymptomatic but with time, many patients develop localized pulmonary hypertension, hemoptysis, and eventually high output cardiac failure. Multidetector computed tomography (MDCT) plays an important role in the diagnosis and planning of definitive treatment by identifying the origin and course of the aberrant artery. Definitive treatment can be surgical (lobectomy or segmentectomy) or endovascular.