Familial polyposis coli (FPC) is an inherited nonsex linked mendelian dominant disease in which there exist at least 100 adenomatous polyps throughout the entire large bowel. Thirteen patients with FPC, of whom 7 were male and 6 female, visited our department between 1966 and 1990. The mean age of the patients was 33.5. Eight of the 13 patients (61.5%) had gastric and/or duodenal polyps, and eight of the 13 patients (61.5%) had colorectal carcinomas at admission. Colectomy with ileorectal anastomosis (IRA) was performed on 2 patients. The functional results were good bowel frequency, good sphincter control and lack of dietary restriction. Clinical experience tended to suggest that the risk of rectal cancer following IRA is extreme and unacceptable, although our two cases were still free of rectal cancer. Since 1980, the total colectomy with mucosal proctectomy and ileal pouch-anal anastomosis was the procedure of choice in our department. Good results were obtained on the six patients on whom this procedure was performed.