Gastrointestinal stromal tumors(GIST)are the most frequent sarcoma arising from gastrointestinal tract. Imatinib and sunitinib are proven to be efficacious against GIST and become standard of care. Moreover several novel molecular-targeted agents are in development. Regorafenib significantly improved progression-free survival in patients with GIST who were treated with imatinib and sunitinib in a randomized phase III trial and is considered a new standard option.