Bilateral xanthogranulomatous pyelonephritis is a rare condition with 7 previously reported cases, all of which required dialysis and/or resulted in death of the patient. We report a case in which bilateral partial nephrectomy was performed successfully for bilateral xanthogranulomatous pyelonephritis. The etiology, symptomatology, radiographic findings and treatment of this rare inflammatory condition are discussed with a brief review of the previously reported cases.