Objective To investigate the diagnosis and clinical value of small bowel CT three-dimensional imaging in Peutz-Jeghers syndrome. Methods 18 cases of PJS confirmed by pathology in changhai hospital were collected, including 9 males and 9 females. All cases underwent small bowel CT three-dimensional examination. The clinical manifestations of these patients were analyzed retrospectively. Results Skin pigmentation were found in 18cases, recurrent abdominal pain as the first symptom in 18 cases, black stool in 8 cases. 6 cases were present with intestinal obstruction, 5 cases with intussusception, 2 cases with intestinal perforation after colonoscopy. 1 case was accompanied by left breast fibroadenoma. There were 12 cases (66.7%) of malignant polyps, 3 cases of inflammatory polyps (16.7%), 2 cases of villous tubular adenoma (11.1%), and 1 case of hyperplastic polyps (5.5%). The location of polyps were 9 cases in stomach, 18 cases in small intestine, and 16 cases in colon. The size of polyps were from 1.0 cm to 4.5cm. The polyps were pedunculated or sessile, round or lobulated, Small strips of vessels entered the polyps from the intestinal wall after enhancement. The typical target sign and double bowel sign were found in the cases with intussusception. Conclusion The Clinical manifestations of Peutz-Jeghers syndrome was special. The small bowel CT threedimensional diagnosis of gastrointestinal polyps with or without intussusception was of high accuracy, and it could be used as a noninvasive examination for following-up.