Extracolonic intestinal manifestations of familial polyposis coli have been described in recent years, but the exact frequency is still unknown. To date the more common sites of extracolonic polyposis appear to be stomach and duodenum with true adenomas being somewhat rare in jejunum and ileum. The authors report a further two cases of ileal polyposis in siblings from a family with familial polyposis coli and conclude that this condition cannot be considered as a disease exclusively of the large bowel. They emphasize that examination of the upper gastrointestinal tract and small bowel must be included in the investigation and follow-up of patients with familial polyposis coli.