Pneumatosis cystoid intestinalis is a rare disease reported in the literature affecting 0.03% of the population. It has a variety of causes and its manifestation may change widely. It usually presents as a marginal finding resulting from various gastrointestinal pathologies. In the acute complicated form of pneumatosis intestinalis, management is challenging for physicians and surgeons. We present a case of a 60-year-old patient who was admitted to our surgical department with a symptomatology suggestive of small bowel occlusion. Computed tomography demonstrated ileal volvulus associated with parietal signs suffering and pneumoperitoneum. An emergent exploratory laparoscopy followed by conversion was performed demonstrating segmental ileal pneumatosis intestinalis secondary to a small bowel volvulus due to an inflammatory appendix wrapping around the distal ileum. Further, detorsion, retrograde draining, and appendectomy were performed because there were no signs of necrosis and the appendix was pathological. The postoperative course was uneventful. This case is exceedingly rare in the literature, because it was featured by the ileal volvulus due to appendicitis.This case report emphasizes the importance of surgical procedures in the management of symptomatic pneumatosis intestinalis associated to an acute abdomen.