A case of central nervous system tumor associated with polyposis of the colon is reported. A review of the literature shows two other such cases, reported by Turcot et al., and concerning two brothers. The symptoms of this association usually begin during puberty. All tumors described untill now are gliomas and colonic polyps have always suffered carcinomatous degeneration. The patient here concerned, a girl aged fourteen, had a spongioblastoma polare of brain stem with multiple polyposis of the colon and carcinoma in some of them. There are no other cases in the family.