Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors that are best classified as sarcomas with variable aggressiveness. They are typically treated with surgical resection and adjuvant tyrosine kinase inhibitors or, for advanced/metastatic GISTs, with tyrosine kinase inhibitors alone. Gastroenterologists are often the first to detect GISTs and are, therefore, tasked with diagnosis and facilitation of early intervention. Diagnostic tools including various imaging techniques such as endoscopic ultrasound (EUS) and EUS-assisted tissue sampling are critical for an accurate diagnosis. In the case of small tumors, EUS-assisted resection or ligation techniques to treat asymptomatic small tumors have been described. This paper reviews current evidence for the diagnosis and management of GISTs, with an emphasis on the role of the gastroenterologist.