Background Portal vein gas is an ominous radiological sign, which indicates a serious gastrointestinal problem in the majority of patients. Many causes have been identified and the most important was bowel ischemia and mesenteric vascular accident. The presentation of patients is varied and the diagnosis of the underlying problem depends mainly on the radiological findings and clinical signs. The aim of this article is to show the clinical importance of portal vein gas and its management in emergency surgery. Methods A computerised search was made of the Medline for publications discussing portal vein gas through March 2008. Sixty articles were identified and selected for this review because of their relevance. These articles cover a period from 1975–2008. Results Two hundreds and seventy-five patients with gas in the portal venous system were reported. The commonest cause for portal vein gas was bowel ischemia and mesenteric vascular pathology (61.44%). This was followed by inflammation of the gastrointestinal tract (16.26%), obstruction and dilatation (9.03%), sepsis (6.6%), iatrogenic injury and trauma (3.01%) and cancer (1.8%). Idiopathic portal vein gas was also reported (1.8%). Conclusion Portal vein gas is a diagnostic sign, which indicates a serious intra-abdominal pathology requiring emergency surgery in the majority of patients. Portal vein gas due to simple and benign cause can be treated conservatively. Correlation between clinical and diagnostic findings is important to set the management plan.