Eight pediatric cases of the diffuse form of xanthogranulomatous pyelonephritis are reported. These patients were selected among 17 with destruction of one kidney. Clinical symptoms were suggestive of a tumor in some instances. However, an infectious etiology was strongly suggested by the presence of a renal lithiasis in seven cases (including five staghorn stones) and of a urinary tract infection in six cases (usually caused by a Proteus). Nephrectomy was performed in every case.