Gastrointestinal stromal tumors (GISTs) are rare tumors of the digestive tract. Despite their rarity, GISTs are of great importance for oncology. Gastrointestinal stromal tumors are one of the first solid tumor types in which specific factors responsible for malignant behavior have been elucidated and for which drugs specifically targeting these factors form the mainstay of treatment in advanced-stage disease. This review addresses several aspects of the current management of GIST as well as some novel developments.