Xanthogranulomatous pyelonephritis (XGPN) is rare form of chronic, usually unilateral, renal infection that involves damage to the renal glomeruli and periglomerular tissue and destruction of the renal parenchyma. Nephrectomy is usually necessary and the prognosis is good if XGPN is unilateral and treated early. XGPN typically affects middle-aged females and is extremely rare in children. We report one case of XGPN in a seven-year-old girl who was operated on at our institution be-cause of lower urinary tract dysfunction and renal insufficiency - the left kidney was removed and the bladder was augmented by means of the remaining ureter. The histopathological evaluation of the removed kidney showed chronic XGPN.