Peutz-Jeghers syndrome (MONDO_0008280, a Monarch Disease Ontology entry) is an autosomal dominant disorder caused by pathogenic variants in the STK11 gene, characterized by hamartomatous polyps in the gastrointestinal tract, mucocutaneous pigmentation and increased risk of GI and extra-GI malignancies. Also known as: Jeghers-Peutz syndrome, PJS, Peutz Jeghers Syndrome, Peutz Jeghers colon polyp, Peutz's syndrome, Peutz-Jeghers polyp of small intestine, Peutz-Jeghers small bowel hamartoma, STK11-related Peutz-Jeghers syndrome.