Three patients with familial polyposis in childhood have been presented. Two siblings were treated via total colectomy and ileoproctostomy with preservation of the ileocecal valve. Residual rectal polyps have remitted spontaneously. Eleven cases of carcinoma complicating familial polyposis in children have been reviewed. Carcinoma may cause death prior to the age of 16 years. Total colectomy and ileoproctostomy is the preferred treatment in childhood. The adjustment to an ileoproctostomy involves minimal postoperative morbidity and few psychological problems. The hazard of carcinoma in the retained rectum is minimal in children.