Lynch syndrome, previously called hereditary nonpolyposis colorectal cancer (HNPCC), can also involve the small bowel. Juvenile polyposis syndrome and PTEN hamartoma tumor syndrome (Cowden disease) are rare inherited hamartomatous syndromes. Intestinal ganglioneuromatosis can occur sporadic or accompany other diseases as multiple endocrine neoplasia type 2B or Cowden disease. For Cronkhite–Canada syndrome, a diffuse digestive, pseudopolypoid, inflammatory process of the GI tract, an autoimmune genesis has been suggested. Although all these polyposis syndromes may involve the small bowel, systematic screening using small bowel capsule endoscopy (SBCE) is not recommended for these patients due to low risk of small bowel malignancy in asymptomatic patients compared to other organs. However, screening for different other types of cancer depending on the specific genotype is suggested. SBCE should be performed in case of unexplained anemia or suspected small bowel bleeding.