Peutz-Jeghers syndrome (PJS) presents in early childhood, and children have a higher risk of intussusception due to a smaller abdominal space than adults. Double-balloon enteroscopy (DBE) has been proven to be a safe and effective method for the diagnosis and treatment of small bowel lesions, but the efficacy and safety of its prophylactic polypectomy in children with PJS need to be determined. Data of 6 children (median age 10.6 years) diagnosed as having PJS from 2018 to 2020 were reviewed. DBE was performed 14 times, and 3 children were successfully relieved of intussusception after DBE treatment. The sizes of the resected intussusception polyps were 50 mm×60 mm, 40 mm×35 mm, and 50 mm×40 mm. Symptoms associated with polyps (abdominal pain, intussusception and obstruction) relieved after DBE in all children. No direct complications such as bleeding or perforation were found during the operation, and no recurrence of intussusception was found during follow-up. It can be seen that DBE polypectomy is safe and effective in the treatment of intussusception caused by PJS.