In a 7-year-old boy, a small-bowel polyp was found intraoperatively as a le
ad point of an intussusception. Histologically, a hamartoma was found and t
he clinical work-up revealed Peutz-Jeghers syndrome (PJS). Additionally, al
l four asymptomatic siblings showed intestinal polyposis. All children in a
family with PJS should be properly investigated. In case of an intussuscep
tion with a polyp in a critical location, a surgical procedure should follo
w.