: Gastro-intestinal stromal tumor (GIST) of the esophagus are rare and account for only 1–2% of all GIST. As a result, there is a lack of clear guidelines regarding their optimal management. Previously mistaken for leiomyomas or leiomyosarcomas, it is relatively recently that their origin from the interstitial cells of Cajal was identified, when their expression of double expression C-Kit and CD34 was discovered. They are submucosal tumors, and their presentation is non-specific, with dysphagia being the most frequent symptoms. They appear as well defined iso or hypoattenuating round tumor with smooth sharp edges on computed tomography, and differ from leiomyoma by being fluorodeoxyglucose (FDG) avid on positron emission tomography. Surgical resection is the mainstay of treatment, however, indications and modality of resection are topic of debate. Due to high morbidity of esophagectomy, minimally invasive solutions are appealing. In this review, we propose a general overview of esophageal GIST, and a discussion the different surgical options.