Peutz–Jeghers syndrome (PJS) is an autosomal dominant polyposis syndrome with high penetrance. It is associated with a germline mutation in the STK11/LKB1 gene in 80–94% of patients and has an incidence of about 1 in 8500 to 1 in 200,000 live births The characteristics of the PJS phenotype include mucocutaneous melanin pigmentation gastrointestinal (GI) polyposis and a predisposition to GI and extra-GI malignancies. GI polyps in PJS have distinct hamartomatous histopathological features, with “frond-like” epithelial lengthening and “arborisation” of a smooth muscle core. Although PJS polyps may occur anywhere within the GI tract, they predominantly occur within the small bowel, particularly the jejunum. Large (≥1.5 cm) PJS polyps in the small bowel frequently result in intussusception. To facilitate early detection of these large PJS polyps and to examine for possible occult small-bowel malignancies, current guidelines recommend that patients should undergo small-bowel surveillance on a biennial to triennial basis starting at 8–10 years of age. Detection of these large PJS polyps within the small bowel by minimally invasive surveillance allows for their pre-emptive removal before an episode of intussusception occurs, avoiding the need for emergency surgery and small-bowel resection. Recently, ischemic polypectomy of these typically pedunculated PJS polyps using clips or detachable snares has been suggested. Surveillance in PJS patients should also include other organs at risk for cancer development.