The authors describe the case of a 7 year old boy who presented an intestinal intussusception due to a large jejunal hamartomatous jejunal polyp. The preoperative diagnosis of Peutz-Jeghers syndrome was based on typical mucocutaneous pigmentation and confirmed by radiological examination including computerized tomography of the abdomen. Following recent data from the literature, a long-term surveillance is justified after diagnosis of this syndrome, since malignant degeneration may occur within the intestinal polyps as well as in other organs.