ObjectiveTo summarize the pathogenesis and epidemiology features of gastrointestinal stromal tumor(GIST), explore its diagnosis and therapy, and analyze its prognosis. MethodThe pertinent literatures about the pathogenesis, epidemiology features, diagnosis, therapy, and prognosis of GIST in recent years were reviewed. ResultsGIST was non-epithelial tumor which derived from interstitial cells of Cajal, was the most common mesenchymal tumor about accounting for 1%-3% in the digestive tract tumor. The median onset age of patients with GIST was 40-60 years. The gastric stromal tumor was about 60% in all the digestive tract tumor. The current consensus statement was that there was a relation between the pathogenesis of the GIST and proto-oncogene c-kit or platelet-derived growth factor receptor alpha(PDGFRα)gene mutation. But the mutations of PDGFRαand c-kit gene did not emerge at the same time in the same patient. The clinical manifestations of GIST were not specific, and the diagnosis mainly depended on endoscope and image technology, the correct diagnosis depended on pathological examination. The treatment of GIST was given priority of surgery and molecular targeted drug therapy, and the prognosis was closely related to risk assessment stratify of GIST. ConclusionsGISTs are mesenchymal tumors that has a potential of malignant transformation, the risk classification criteria for aggressive clinical course of primary GIST is an important indication for guiding the clinical therapy and prognostic evaluation. Further research would be needed in prevention, diagnosis, treatment, and relapse prevention of GIST.