A 21-year-old woman with fever and dyspnea was admitted to our hospital. X-ray examination of this patient showed abscess forming in the right lower lobe of the lung. Aspergillus fumigatus was detected by percutaneous lung biopsy. She had repeated pneumonia since her childhood, so congenital lung disease was suspected. Aortic angiography showed the flow of an aberrant artery originated from the right inferior phrenic artery into the right lower lobe. Preoperative diagnosis of the patient was lung sequestration associated with aspergillosis. The operative procedure included right lower lobectomy with ligation of the aberrant artery. Postoperative diagnosis was intralobar sequestration of Pryce III type which was infected with aspergillus. Intralobar sequestration associated with aspergillosis is very rare, perhaps this is the third case in the literature of Japan.