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Pneumatosis cystoides intestinalis (PCI) is a disease characterized by the presence of multiple air-filled cysts in the intestinal submucosa or subserosa. Although PCI is sometimes observed in the adult population, it is rare in children. Here we report the case of a disabled child who developed PCI complicated by intussusception while receiving treatment for chronic bronchitis in our hospital. A 4-year-old male patient showed bloody stools and decreased percutaneous oxygen saturation (SpO2) on the third day of hospitalization for treatment of an acute change of chronic bronchitis. He had been treated for severe psychosomatic disorders induced by hypoxic ischemic encephalopathy attributed to the complete transposition of the great arteries Type I. He had also suffered from symptomatic epilepsy and chronic hard constipation, and had been fed by gastrostomy. A physical examination showed developmental delay. His height and weight were 91 cm ( 3.2 SD) and 12.6 kg ( 1.9 SD) respectively. An elastic soft mass was palpated on the right upper abdomen. A laboratory analysis showed the following: hemoglobin, 14.8 g/dL; leukocyte count. 14100/μL; and C-reactive protein, 0.23 mg/dL. Non-specific Escherichia coli was detected in the stool culture. Chest X-ray showed low permeability on both lungs and abdominal radiograph showed dilated intestines and a large amount of feces. Ultrasound revealed the target sign in the right upper abdomen and intussusception was diagnosed (Fig. 1a). Non-operative reduction of the intussusception was attempted with air and gastrografin enema (Fig. 1b) but failed and operative reduction was selected. At laparotomy, colocolic-type intussusception was found in the ascending colon and manual reduction was successfully performed with Hutchinson’s maneuver. After the reduction of the intussusception, a submucosal mass (size: 3x4 cm, height x width) composed of air-filled multiple cysts was observed in the ascending colon, and was considered to be the pathological lead point of the intussusception (Fig. 1c). The multiple cystic mass was resected with the adjunctive segment of the colon, and a pathological examination of the resected specimen revealed PCI of the ascending colon (Fig. 1d). At 2 years after surgery, the patient had no recurrence of PCI or intussusception. Pneumatosis cystoides intestinalis is a special type of pneumatosis intestinalis (intramural air) characterized by the presence of multiple air-filled cysts in the intestinal wall. It is sometimes observed in neonates or elderly patients. Pneumatosis cystoides intestinalis is more frequently located in the colon than in the small intestine. It tends to be observed among geriatric patients, who sometimes suffer from chronic constipation and whose intestinal walls tend to be fragile. In the rare reports of PCI in children, the patients have frequently suffered from neurological, gastrointestinal, and respiratory impairment. Pneumatosis cystoides intestinalis in children is frequently complicated by chronic bronchitis and enterocolitis, and children with PCI are often fed by gastrostomy. The causes of PCI remain unknown. Elevated intraluminal pressure, pulmonary disease, bacterial gas production, malnutrition, chemotherapy, and connective tissue diseases or other conditions have been proposed as causes. Symptoms of PCI are non-specific, and include abdominal pain, abdominal distention, nausea and vomiting, bloody stool, constipation, or diarrhea. Pneumatosis cystoides intestinalis is usually benign and asymptomatic, but it is sometimes complicated by bowel obstruction, volvulus, intussusception, and bowel perforation. Radiological images show radiolucent shadows in the bowel wall on abdominal X-ray or computed tomography (CT). Computed tomography is also useful for identifying co-existing diseases, such as perforation, obstruction, and neoplasm. Most cases are successfully treated with conservative management, such as antibiotics, high-flow oxygen and / or hyperbaric oxygen treatment. Surgical treatment is performed in rare cases involving patients who do not respond to conservative management, and operation is required in rare cases with bowel perforation or non-operative reduction of intussusception. In our case, the patient had severe motor and intellectual disabilities, cardiac malformation, chronic respiratory failure Correspondence: Yoshie Matsubara, MD, Department of Pediatrics, Kyushu University Hospital, 3-1-1, Maidashi, Higashi-ku, Fukuoka-shi, Fukuoka-ken 812-8582, Japan. Email: [email protected] The addresses of the author’s affiliated institutions at which the work was carried out: NHO Kokura Medical Center. Received 26 November 2019; revised 13 February 2020; accepted 25 February 2020. doi: 10.1111/ped.14219