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Pulmonary sequestration is an unusual malformation. It consists of a nonfunctioning mass of lung tissue that shows no normal continuity with the tracheobronchial tree and derives its arterial blood supply from the systemic circulation. The thoracic and abdominal aortas are the most common sites of origin of the abnormal nutrient branches. Arterial supply of a sequestration from the coronary circulation is extremely rare. We present a case of a right middle lobe sequestration deriving its branches from the left coronary artery.