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Pneumatosis intestinalis is an unusual finding that can exist as a benign entity but can indicate a life threatening emergency and the need for surgical intervention. The existing information still confuses the medical community. Considering the wide range of causes and the outcomes of this entity, physicians are frequently faced with a dilemma in identifying those patients who actually require surgery. The understanding of pneumatosis intestinalis with an early recognition and appropriate management is of great importance. This article presents a comprehensive review of scientific literature about the subject, trying to capture in a simple way the existing knowledge about a complex entity.