[Congenital xanthogranulomatous pyelonephritis]

The authors describe a rare case of this disease that has clinically manifested in a 12-days-old boy. At the age of 1.5 months a right-side nephrectomy for a supposed Wilms' tumor has been performed. Histologic examination has diagnosed congenital xanthogranulomatous pyelonephritis. The authors put forward a hypothesis on the existence of primary forms of this condition not associated with persistent infections or urinary tract obstructions.