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A case of emphysematous pyelonephritis is presented and a review of literatures is made. A 67-years-old woman with diabetes mellitus, complained of severe left flank pain and high fever and was referred from the department of gastroenterology. A computed tomography film demonstrated gas in the left renal parenchyma and a diagnosis of emphysematous pyelonephritis was made. She was in a state of shock and died after 4 days of conservative treatment. Emphysematous pyelonephritis is a rare, life-threatening disorder and 85 cases in the world have been reported to date. Our case is the 18th one in Japan. Its etiology, symptomatology, treatment and prognosis are discussed.