A 69-year-old man presented with intestinal obstruction and peritonism. Chest and abdominal X-rays showed massive pneumoperitoneum with large bowel obstruction (Figs. 1, 2). Exploratory laparotomy revealed closed loop obstruction secondary to sigmoid volvulus with extensive pneumatosis cystoids intestinalis of non-dilated small bowel (Figs. 3, 4), without evidence of perforation. We postulate that rupture of a submucosal cyst in the small bowel resulted in pneumoperitoneum. Total colectomy was performed, with ileocecal anastomosis and defunctioning ileostomy. Histology confirmed pneumatosis cystoides intestinalis of small bowel without full-thickness perforation (Figs. 5, 6).

Fig. 1
figure 1

Erect chest X-ray showing pneumoperitoneum

Fig. 2
figure 2

Supine abdominal X-ray showing grossly dilated large bowel and multiple, clustered, small gaseous lucencies in the right lower abdomen, probably caused by air within the bowel wall, characteristic of pneumatosis intestinalis

Fig. 3
figure 3

Intraoperative image showing extensive pneumatosis intestinalis of the non-dilated distal small bowel

Fig. 4
figure 4

Gross image showing cystic air bubbles in the submucosa

Fig. 5
figure 5

Low-power microscopic view showing large cystic lesion in the submucosa

Fig. 6
figure 6

High-power microscopic view showing foreign body giant cells lining the cystic spaces