Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder characterized by intestinal hamartomatous polyps in association with mucocutaneous pigmentations. Main symptoms of PJS in childhood are abdominal pain, obstruction, intussusception, and bleeding from hamartomatous polyps. PJS carries a high risk of gastrointestinal cancer with advancing years. Although, intussusception has been reported as a well-known complication of PJS, recurrent intussusception as an alarming finding in a patient with normal gastrointestinal endoscopy is uncommon. A 7-year-old boy who had recurrent intussusception episodes and diagnosed with PJS histopathological confirmation after surgical excision of involved bowel segments is presented to discuss the clinical features and treatment options of recurrent intussusception as a presenting finding of PJS.

Keywords: Peutz-Jeghers syndrome, cancer, child, intussusception