Abstract Polyps of the small bowel are rare compared to those of the colorectum. A correct histopathological diagnosis is crucial for the choice of subsequent treatment. This article reviews the most frequently found and some rare but distinct polyps and polyp-like lesions in the small intestine. Adenomas are the most commonly found polyps in the small intestine. Other polypoid lesions include Brunner gland hyperplasia, Brunner gland hamartoma, periampullary myoepithelial hamartoma and pyogenic granuloma. Adenomas are usually found in the distal portion of the duodenum, whereas, Brunner gland hamartoma and inflammatory polyps are noted in the proximal portion of the duodenum. The rare but distinct Peutz–Jeghers polyp and juvenile polyp are reviewed, including the associated hereditary autosomal dominant syndromes (i.e. Peutz–Jeghers and juvenile polyposis syndrome) of which these lesions are the phenotypic hallmarks. Finally, an extremely rare polyposis syndrome with unknown aetiology, i.e. Cronkhite–Canada syndrome, is described with documentation.