Abstract: As the most common mesenchymal tumor of gastrointestinal tract，gastrointestinal stromal tumor（GIST） has been paid more and more attention. In the recent 10 years，with studies on molecular mechanism of GIST，and emergence of molecular targeted drugs represented by imatinib mesylate，traditional treatment concepts of GIST have been updated. Surgical resection combined with molecular targeted drugs is becoming the treatment mode for GIST. Molecular targeted therapy has significantly improved the prognosis and prolong the survival of GIST patients. However， for different stages of GIST，many questions on how to apply the targeted drugs reasonably still remain to be answered. This article summarizes the latest progress on the perioperative treatment of GIST and systemic therapy for advanced GIST.