There are few reports of gastrointestinal stromal tumors (GISTs) in the esophagus. The authors report a patient with an esophageal GIST that was found incidentally during an endoscopy. The endoscopy revealed a 1 cm sized mass with a granular surface at the 32 cm site from the upper incisor. Endoscopic ultrasonography revealed the tumor to be located in the muscularis mucosa of the esophageal wall. Histologically, the tumor consisted of spindle cells, with no mitotic index, that were immunoreactive for KIT and S-100. The tumor was diagnosed as a gastrointestinal stromal tumor with neural differentiation (GINT). An endoscopic mucosal resection was performed and the patient has been on routine follow up at the out patient department for three months.