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Small bowel and duodenal polyps are commonly seen in the rare polyposis syndromes, where they may be a major clinical problem and may be a cause of significant morbidity and indeed mortality. Sporadic polyps at these sites, however, are uncommon. This chapter reviews the current level of understanding of such polyps and outlines current approaches to surveillance, as well as endoscopic and surgical interventions used to treat them. Data are reviewed according to polyp subtype and then by the syndromes in which such polyps occur. The best data are available for familial adenomatous polyposis and so there is a focus on this syndrome which, given its incidence, will be the condition to which clinicians are most likely to be exposed.