Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract, the majority of which is KIT (CD117) positive. In this case report, we describe a case of recurrent and metastatic GIST who presented with hepatic and brain metastases. Despite the patient's GISTs was negative for c-Kit (CD 117), he responded to imatinib mesylate (Glivec) treatment with complete resolution of his liver and brain lesions. The patient has been and still in complete remission for 18 months of follow-up.