DOI: 10.36347/sjmcr.2017.v05i03.002

Pages: 138-140

A rare syndrome presenting with muco-cutaneous pigmentation with hamartomatous polyposis of intestine associated with increased risk of gastrointestinal and other systemic malignancies popularly termed as Peutz- Jeghers Syndrome present as varied course of symptoms, some report to dermatologist for pigmented oral macules, some report in emergency with acute abdomen while others are diagnosed incidentally. We report a 6 years old male child brought to emergency department with acute abdomen subsequently diagnosed with intussusception caused by a hamartomatous polyp which later on combined with pigmented macules on buccal mucosa ended up as an autosomal dominant syndrome.