Volvulus and intussusception are rare conditions in children. We describe an unusual case of intussusception due to a solitary Peutz-Jeghers type hamartomatous polyp complicated by volvulus, which occurred in an 11-year-old girl. A laparotomy allows to successfully treat the pathology. The postoperative course was favourable. We discuss the clinical findings and the values of the preoperative instrumental diagnosis. The literature is reviewed. Identifying a midgut volvulus, as complication of a small bowel intussusception, during the diagnostic phase could help in choosing the most appropriate surgical approach.