 GISTs are uncommon visceral sarcomas that arise predominantly in the gastro-intestinal tract. Most GIST cells are positive for c-kit CD117, a cell surface antigen corresponding to the Stem Cell Factor SCF receptor. The receptor has an intracellular tyrosine kinase TK joined by a juxtamembrane domain. It is hypothesized that all malignant GIST cells harbor a mutation of c-kit, resulting in the activation of c-kit and cell division and tumour growth. Drugs that can selectively inhibit TKs are likely to be of benefit in GISTs. Masitinib AB1010 is a TK inhibitor, selectively and effectively inhibiting c-kit. Imatinib is also a TK inhibitor indicated in the treatment of GIST. It might be associated with side effects and patients might develop a resistance to treatment over time. Based on pre-clinical and clinical studies, masitinib AB1010 can be considered as a good candidate in the first line treatment of patients with GIST. 