Intralobar pulmonary sequestration combined with aberrant systemic a tery-pulmonary vein fistula is rare congenital malformation. Authors experienced a case of intralobar pulmonary ssequestration combined with arteriovenous fistula in left lower lung. The patient was 8 year-old male whose complaint was dyspnea on exertion. The continous mumur was heard over the left posterior chest wall. The chest film showed multiple tortuous branching tubular shadows in theleft lower lung field and dilated pulmonary vein at the infrahilar area. Thoracic aortogram showed that aberrant systemic artery originatded form lower thoracic aorta and the arterial blood drained into left atrium directly through dilated left lower pulonary vein. Left pulmonary arteriogram revealed that there was no vascular supply in the posterior basal segment of left lower lobe and enveloped by the same visceral pleura. A segmentectomy of left lower lobe with ligation of aberrant vessels was performed.