Emphysematous pyelonephritis (EPN) is a rare but serious infectious disease caused by anaerobic bacteria that is characterized by necrotizing renal parenchymal infection and has a high mortality rate. The initial management of EPN includes resuscitation, antibiotics, management of any diabetes, and minimally invasive procedures, such as percutaneous drainage. Surgery is required in the most severe cases. Here, we describe a male patient with type 2 diabetes who presented with fever and abdominal pain and was diagnosed with EPN based on laboratory results and a computed tomography (CT) scan showing distension of the left kidney, several small air bubbles in the perirenal space, and thickening of the perinephric fascia. A subcutaneous insulin infusion and meropenem were administered. Repeat CT imaging 13 days later showed gas and necrotic tissue in the renal parenchyma. Therefore, CT-guided renal puncture was performed, and Escherichia coli was cultured from the drained pus. Antibiotic treatment was continued for 20 days after admission when the patient's kidney function and clinical symptoms had significantly improved. Sixty days after diagnosis, left nephrectomy was performed. The perinephric abscess was under high tension, and the left kidney was very soft. Histopathological examination revealed severe inflammation and necrosis of the renal pelvis, with fibrosis. The patient recovered well after surgery and remained alive 5 months later. EPN is a very rare disease with a high mortality rate and is more likely to occur in patients with diabetes. Early diagnosis and treatment of the infection are extremely important. For patients in whom gas and/or necrosis continue to accumulate after conservative treatment, we recommend nephrectomy.