This clinical report presents a 45-year-old female patient with acute kidney injury, hepatic dysfunction and urosepsis without past history of any illness related to urinary system. Laboratory investigations showed Hb 15.6 gm/dl, neutrophilic leukocytosis, thrombocytes 9000/mm 3 , ESR 90 mm/h, plasma thromboplastin time (PTT) 48 seconds (control 32 seconds), INR 2, and fibrinogen degradation product 7500 ng/ml (normal <250 ng/ml). Serum creatinine was 450 µmol/L (5.1 mg/dl), direct bilirubin 68.40 µmol/L, alanine aminotransferase 1.6 mmol/L and albumin 25 gm/L. Urinalysis revealed leucocyturia, hematuria without casts and proteinuria. Abdominal plain x-ray revealed left-sided radio-opaque shadow at the ureteric line, left uretero-hydronephrosis with thickened cortex and left ureteric stone, but normal right kidney. Computerized tomography of abdomen showed a large left kidney (13.5 cm) with totally distorted architecture, multiple enhancing and nonenhancing sectors, thickening of renal capsule, moderate dilatation of renal pelvis, dilated ureter and no passage of contrast in the ureter. The patient was treated with a 2-week course of Ceftriaxone and Aztreonam and pre-operative session of hemodialysis before the performance of a left-sided nephrectomy. Gross examination revealed adherent capsule, granular cortex covered with purulent exudates, no demarcation between cortico-medullary junction, and the cut surface showed minute abscess and necrosis with markedly dilated pelvis. Light microscopic examination showed fibrosis and inflammation of parenchyma with vacuolized histiocytes foam cells surrounding necrotic areas confirming the diagnosis of xanthogranulomatous pyelonephritis. The patient was discharged after 35 days of hospital stay with normal renal and hepatic function.