Pneumatosis cystoides intestinalis is an uncommon condition characterized by the presence of multiple gas-filled cysts within the gastrointestinal wall. This lesion occurs in association with a large variety of gastrointestinal and non-gastrointestinal conditions. Herein, we describe a case of pneumatosis cystoides intestinalis of the small intestine that developed in a 31-year-old man with a history of duodenal ulcer and pyloric stenosis. Emergency exploro-laparotomy was done due to a suspicion of ulcer perforation. Primary closure for duodenal ulcer perforation and segmental resection of ileum were done. Resected ileum showed diffuse sponge-like changes in the wall. Microscopically, variable-sized cystic lesions, lined by flat or multinucleated giant cells, were noted throughout the intestinal wall.