The Peutz-Jeghers polyp is an unusual type of hamartomatous polyp; its characteristic feature is a smooth muscle core arising from the muscularis mucosae and extending into the polyp. Peutz-Jeghers polyps vary in size and shape; are found in the stomach, small bowel, and colon; and are usually multiple. Peutz-Jeghers syndrome is an inherited condition that often remains undiagnosed until after the polyps are identified, despite mucocutaneous pigmented lesions on the lips and mouth of children or young adults. In the past, standard therapy involved removal of the polyps that produced intussusception, but now endoscopic removal of all polyps is recommended. The polyps are not premalignant, but a definite association exists between Peutz-Jeghers syndrome and gastrointestinal carcinoma. Evidence shows that the syndrome is associated with an increased risk of extraintestinal malignancy, especially carcinomas of the pancreas, breast, and reproductive organs.