Abstract: The biological behavior of esophageal gastroiontestinal stromal tumors is worse than that of gastrointestinal stromal tumors (GIST), so surgical treatment is the main treatment. The operative methods include endoscopic therapy, tumor enucleation and partial resection of the esophagus. Endoscopic treatment methods include endoscopic submucosal dissection (ESD) and submucosal tunnelling endoscopic resection (STER). With the development and maturity of minimally invasive technology, surgical treatment of esophageal GIST is worth further discussion based on the principle of minimal trauma and the best curative effect. In recent years, molecular targeted therapy, including neoadjuvant therapy and postoperative adjuvant therapy, has become a major advance in the treatment of GIST. The success of molecular targeted therapy opens a new era in the molecular targeted therapy of cancer.