Intestinal hamartomatous polyposis, mucocutaneous macules combined with an increased risk of carcinomas of the gastrointestinal tract, breast, thyroid and pancreas define the so called Peutz-Jeghers syndrome. This inheritable disease occurs normally within the first three decades of life and manifests symptoms like abdominal pain, rectal bleeding, anemia or obstruction. In the facial region it shows black-to-brown spots, mostly located in the perioral area and on the lower lip but also possible on the oral mucosa. The macules themselves are benign, less than 1mm in diameter and likely to disappear during adolescence. Life-long cancer screening, as well as gastrointestinal polyp resolutions are recommended.