A case of intralobar pulmonary sequestration with three aberrant arteries was reported. The patient was a 26-year-old man. His chest film showed an abnormal shadow suspected of a pulmonary sequestration in the left lower lobe of the lung. Computed tomography of the chest revealed that the aberrant arteries originated from the descending aorta and circulated the emphysematous area of the left lung. Angiography clearly demonstrated three aberrant arteries and three veins draining into the hemiazygos and lower pulmonary veins. Pulmonary sequestration was diagnosed and then resected. Microscopically, the resected lung had emphysematous change, multiple cysts and winded vessels. Aberrant arteries were the elastic type of vessels and accompanied with atherosclerosis. According to the review of 139 Japanese cases of intralobar pulmonary sequestration, 7% of all patients had more than two aberrant arteries. Moreover, only 3% of patients had veins draining into the systemic vessels. In many cases the thin aberrant arteries were clearly demonstrated by angiography and the draining veins were detected. We consider that angiography is necessary to show definitely the aberrant arteries and drainage veins.