Xanthogranulomatous pyelonephritis (XGP) is a chronic, destructive, granulomatous inflammation of the renal parenchyma leading non-functioning kidney. Contrast-enhanced computed tomography is the most useful diagnostic method. A 6-year-old female patient admitted to us with complaints of abdominal pain, fever, fatigue, weakness. Recurrent urinary tract infection was present in her history. The patient’s urine specimen revealed leukocyturia, hematuria, positive for nitrite and bacteria, white blood cell: 12.7x103 /microl, hemoglobin:6.2 g/dl and CRP: 101 mg/dl. In the urinary system USG imaging, the contours of the left kidney were irregular and lobulated, and medullary punctate echogenicity and calcifications were present. Contrast-enhanced abdominal computed tomography showed increased left kidney size and opacities suggestive of stone in the collecting system; pararenal area was heterogeneous. Although the patient's gentamicin treatment was completed in 7 days and meropenem treatment in 14 days, acute phase reactants did not regress. No activity uptake was observed in the left kidney lodge in renal cortical scintigraphy. Left total nephrectomy was performed with the diagnosis of non-functioning left kidney and chronic pyelonephritis. Kidney biopsy material were reported as XGP. XGP is a rare and aggressive cause of chronic pyelonephritis with serious consequences such as nephrectomy requirement.

Keywords: Chronic pyelonephritis, Pyelonephritis, Urinary tract infections, Xanthogranulomatous pyelonephritis, XGP