Xanthogranulomatous pyelonephritis is a clinico-pathological entity that is gaining awareness amongst urologists worldwide. It is an unusual chronic inflammatory lesion involving the kidney that destroys renal parenchyma and may mimic renal carcinoma. It is usually seen in middle-aged women and is associated with urinary tract infections and urinary calculi. Most reports and reviews of this condition come from the West; this study reviews the incidence and presentation of the condition in a Malaysian population.