A 21-year-old man was admitted to our hospital because of abdominal pain. Abdominal ultrasonography showed the target sign in the right lower abdomen and abdominal enhanced computed tomography showed a fatty layer in the ascending colon. A diagnosis of intussusception was made, which was reduced by a gastrografin enema. The patient recovered and was discharged. Colonoscopy showed elevated lesions like submucosal tumors in the cecum and ascending colon. Then, the patient was referred to us again with intussusception, and we performed ileocecal resection. The resected specimen showed soft polypoid lesions in the cecum and ascending colon, the surface of which showed multiple gas-filled cysts. Histopathological examination revealed pneumatosis of the submucosal layer. A diagnosis of pneumatosis cystoides intestinalis (PCI) was made. The postoperative course was good and the patient was discharged 9 days after the operation. PCI is a relatively rare disease. We report a case of PCI with recurrent intussusception over the short term and a review of the relevant literature.