Total colectomy with ileoproctostomy was performed in 32 members of a family spanning three generations and ranging in age from 10 to 54 years. In seven of these patients (22 percent) carcinoma developed in the retained rectum over a median follow-up period of 14 years. This high incidence of rectal carcinoma has demanded reevaluation of treatment recommendations in patients with polyposis coli. Ten patients aged 7 to 30 years have undergone total abdominal colectomy with ileoanal endorectal pull-through since 1980. All were one stage procedures without reservoir construction. Within 3 months the patients all had good control with 5 to 10 semiformed stools daily and had resumed normal activities. Follow-up date shows adequate dilatation of the distal ileum and no evidence of polyps. Total colectomy and ileoanal endorectal pull-through are effective treatment for familial polyposis in patients of all ages. It should be considered the primary procedure in new patients and an excellent method of converting those patients who have ileoproctostomy to a safer situation.