Pneumatosis intestinalis is an uncommon finding that may indicate the presence of several alarming pathological conditions, including bowel ischemia, that require urgent surgical intervention. We report the case of a 51-year-old man with celiac disease who underwent resection of a large duodenal adenocarcinoma. Although he initially recovered rapidly from his procedure, he subsequently developed abdominal distention and leukocytosis. Abdominal imaging revealed extensive small bowel pneumatosis and pneumoperitoneum. Emergent surgical exploration revealed only bowel wall air cysts and dilated bowel but failed to demonstrate any intra-abdominal pathology. The patient recovered uneventfully and was discharged without any further complications or recurrence of symptoms. We review the current literature on the rare finding of pneumatosis intestinalis in the setting of celiac disease. In all reported cases, even when pneumatosis is accompanied by pneumoperitoneum, these alarming findings have proved to be of "benign" origin, that is with no evidence of bowel ischemia, perforation, or peritonitis. The available evidence suggests that pneumatosis in the setting of celiac disease may reflect the dissection of intraluminal gas into the inflamed bowel wall without accompanying intra-abdominal pathology. We conclude that pneumatosis intestinalis, even with accompanying pneumoperitoneum, does not uniformly mandate surgical exploration in patients with celiac disease.