<p>Peutz–Jeghers syndrome (PJS) is a rare autosomal dominant disorder characterised by mucocutaneous pigmentation and multiple hamartomatous polyps of the gastrointestinal tract. Intussusception is the most common and potentially life-threatening complication, often presenting in childhood or young adulthood and requiring emergency surgical intervention. Although patients with PJS have a significantly increased lifetime risk of malignancy, malignant transformation within hamartomatous polyps presenting concurrently with intussusception is exceedingly rare. We report a 17-year-old gentleman who presented with recurrent abdominal pain and features of subacute intestinal obstruction. Imaging revealed ileo-ileal and ileo-caecal intussusceptions with multiple gastrointestinal polyps. Emergency exploratory laparotomy with right hemicolectomy was performed. Histopathological examination demonstrated well-differentiated adenocarcinoma arising within a hamartomatous polyp. The postoperative course was uneventful, and the patient was started on adjuvant chemotherapy with planned endoscopic surveillance. This case highlights the importance of early surgical intervention in PJS-related intussusception and underscores the need for vigilant lifelong surveillance due to the risk of malignant transformation.</p>