A case of pneumatosis cystoides intestinalis with pneumoperitoneum and volvulus complications is reported. After careful examination of the literature, etiopathogenic, pathophysiological and clinical features of the disease are analyzed by the Authors, also in the light of recent findings. Major diagnostic and therapeutic implications are discussed regarding possible complications requiring a different therapeutic approach. PCI, a lesion which can be benign, primary or secondary in many conditions, may be characterized by the presence of spontaneous pneumoperitoneum which only requires a careful, but not aggressive therapy, unlike other complications such as volvulus or intestinal occlusion which, instead, require timely surgical action. The Authors describing the therapy adopted, highlight the positive role of the enteral nutrition (NE) and oxygen therapy in the clinical course of the disease.