A case of retroperitoneal fibrosis with ureter compression is reported. Clinical picture and course were determined by the presence of an additional tumor (astrospongoblastoma) of the pons. Extraretroperitoneal tissue changes found at post mortem examination were shown histologically to be foreign tissues of the same type deposited in the retroperitoneal space (disseminated xanthofibrogranuloma). Possible connections between the disseminated xanthofibrogranuloma, the pontine tumor and an albinism also present and multiple nevocytic nevi of the skin are discussed.