Intralobar pulmonary sequestration is a rare congenital malformation characterized by a cystic portion of the lung that derives its arterial blood supply through aberrant vessel directly of systemic circulation. Two forms of pulmonary sequestration occur; intralobar sequestration is usually contained within the visceral pleura of a pulmonary lobe and its venous drainage to the pulmonary venous system. We presented on case of intralobar pulmonary sequestration with A-V fistula. The patient was 8 years old male and chief complaints are mild fever and exertional dyspnea. Aortogram revealed an aberrant artery originated from thoracic aorta just above the diaphragm and its vessel directly communicated pulmonary vein in sequestrated segment. At time of operation, sequestrated lobe measuring 6x5x5cm, well demarcated at the lower lobe was noted. Aberrant, measuring 1.0cm in diameter and 1cm in length and directly connected pulmonary vein. After division and ligation of the aberrant artery, only excision of sequestrated lobe was performed and complication is not during postoperative course.