Abstract

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder, which manifests as mucocutaneous hyperpigmentation and, intestinal and extraintestinal polyps. The classic triad of abdominal pain, mass and jam-like stools are not found commonly. On clinical examination, a strong suspicion of PJS should be made if patients below 18 years, present with mucocutaneous hyperpigmentation and intestinal obstruction. We report a case of a 16-year-old woman who presented to the surgical emergency with a 1-day history of acute pain in abdomen, in right iliac fossa and features of intestinal obstruction. A thorough history and clinical examination supported by diagnostic imaging should be done in suspicious cases for prompt diagnosis and appropriate treatment.