This case is a 30-year-old male who was indicated abnormal shadow in the left upper lung field by chest X-ray film. The aortography was carried out because pulmonary sequestration was suspected by chest CT-scan etc. From the result, rare pulmonary sequestration in the left upper lung field was diagnosed because patterns with inflow of an abnormal artery from descending aorta into the left upper lung field and perfusion of it into the upper pulmonary vein were observed. The wedge resection was performed. The specimen was slightly hard on the whole, and many cartilages or multiple nodules were observed on the cut surface. Histopathologically, normal pulmonary structure was disappeared, and hyperplasia of the lymphatic nodule was remarkably observed in the interstitium, and cystic wall was covered by ciliated cylindrical epithelium.