Although only 10-30% of gastrointestinal stromal tumors (GISTs) are clinically malignant, all have some degree of malignant potential. The management of high risk patients should be evidence based. However, prospective data and a consensus for guidelines concerning the screening of asymptomatic high risk patients and surveillance following multidisciplinary treatment do not exist. This review provides an overview of GIST, with an emphasis on the available data regarding screening and surveillance of certain populations with GISTs.