Gastrointestinal stromal tumors (GIST) are the most common digestive mesenchymal tumors. GIST characterized by over-expression of the tyrosine kinase receptor KIT. GIST span a wide clinical spectrum from benign to highly malignant. Surgery is the only curative treatment for GIST. Low malignant GIST have an excellent prognosis after surgical treatment (5 years survival rate > 90%). Highly malignant GIST have an extremely poor prognosis even after surgical resection (median survival < 12 months). The development of tyrosine kinase inhibitors has changed the management of unresectable GIST. One of them, imatinib mesylate, has been proved to improve survival of metastatic GIST. This paper reviews literature data on GIST.