Peutz-Jeghers syndrome is an autosomal dominant hamartomatous polyposis involving gastrointestinal tract associated with mucocutaneous hyperpigmentation involving lips, buccal mucosa ,hand and feet and predisposing the patient to various extraintestinal and intestinal cancers.This article includes case report of a 12 year female diagnosed with intussusception due to Peutz-JeghersSyndrome. This patient underwent emergency exploratory laparotomy followed by resection of about 30 cm necrosed , gangrenous ileum about 50cm proximal to ileocecal junction and then ileoileal anastomosis performed . This patient had pigmented hypermelanotic macules over lower lip ,buccal mucosa and face. This case was thoroughly examined and found to be a rare case of intestinal obstruction in paediatric patients. Key words: Peutz Jeghers syndrome; intussusception; hamartomatous polyposis