Based on four new cases of emphysematous pyelonephritis, the authors review a total or 59 interpretable cases of this rare disease reported in the literature. The general features of this condition are as follows: diabetic women are most frequently affected, the clinical context is generally very serious, the diagnosis is based on X-ray findings, radiating or crescent-shaped radiolucent gas images are observed with a non-functioning kidney on intravenous pyelography. CT scan was performed in two cases and confirmed the presence of air in the renal parenchyma and provided a better evaluation of the extension into the perirenal space. The only effective treatment is nephrectomy which has been able to improve the prognosis of this very serious disease, especially as the characteristic necrosis observed on histology leaves no hope of functional recovery.