The incidence of small-bowel polyps is very low in the general population, but it is significantly increased in hereditary polyposis syndromes such as familial adenomatous polyposis (FAP). FAP is caused by a pathogenic variant in the APC gene, a classical tumour suppressor gene. Colorectal polyposis is the most striking clinical manifestation, and when associated with more than 100 colorectal polyps, leads in almost all patients to colorectal carcinoma unless colectomy is preformed. Prophylactic colectomy is recommended after adolescence at a time based upon the colorectal polyposis burden. Small bowel capsule endoscopy (SBCE) seems to be safe and effective in diagnosing jejunal and ileal polyps in FAP patients, but the clinical relevance of detecting these polyps is yet unclear. SBCE cannot replace upper endoscopy as gastric and duodenal polyposis is near universal and requires surveillance. In FAP patients without duodenal adenomas, SBCE is not helpful, as the incidence of jejunal and ileal polyps is very low.