Abstract: Gastrointestinal stromal tumor (GIST) is the most common type of gastrointestinal mesenchymal tissue tumors, originated in Cajal cells or gastrointestinal pacing cells. It can grow in any part of the gastrointestinal tract, where the stomach and small intestine are multiple, followed by colorectal. The pathogenesis of GIST is associated with activation of c-kit and platelet-derived growth factor receptor-alpha (PDGFRA). Due to its lack of characteristic clinical manifestations, the final diagnosis depends on pathology, and the pathologic diagnosis depends on morphological and immunohistochemical results. The feasible genetic analysis that tests the expression of CD117 and DOG1 will contribute to the diagnosis of special genotype GIST if necessary. At present, surgery combined with targeted therapies is the popular solution for GIST. With the development of new surgical instruments, the laparoscopic surgery has also been gradually recognized by the people in recent years. Imatinib, as a tyrosine kinase inhibitor, can specifically inhibit the expression fo kit, PDGFRA and bcl-al, it had been approved for use in 2001. Sunitinib, when compared with Imatinib, is with more targets, which is applied as the second-line drugs for GIST. The action mechanism of the two drugs is similar. Molecular targeting drugs, represented by Imatinib, is effective in the treatment of GIST and improving the prognosis of patients, which represents milestone significance in molecular targeted therapy of tumors. However, with the increase of drug time, GIST resistance gradually becomes one of the important factors affecting the prognosis of patients with GIST. The pathogenesis of GIST is complex and the variety of gene mutations is diverse, so it is more important to develop specific treatment regimens based on the individual patient's condition. Risk grading is widely used to determine prognosis and to guide molecular bio-targeted therapy. In this paper, the progress of diagnosis and treatment of gastrointestinal stromal tumors at this stage is reviewed.