The report retrospectively analyzed the clinical data and pathological characteristics of one case of pneumatosis cystoides intestinalis of the sigmoid colon complicated with intestinal obstruction confirmed by pathological diagnosis, reviewed other cases from the related literature, and summarized the pathogenesis, pathological characteristics, clinical manifestations, diagnosis, and treatment progress. Pneumatosis cystoides intestinalis (PCI) is a rare disease of digestive system characterized by the presence of multiple gas-filled cysts in the intestinal submucosa and/or subserosa and/or muscularis. PCI was often missed diagnosis or misdiagnosed in clinic because it is usually asymptomatic or with no specific clinical manifestation. Strengthening the most understanding of PCI in clinic can effectively improve the accuracy of diagnosis with the improvement of inspection methods in recent years.