First described in 1926 by Abrikossoff, a granular cell tumor is a benign lesion and commonly involves the skin, connective tissue, and breast, but rarely the esophagus. The origin of granular cell tumors is controversial. The tumor is thought to arise from a Schwann cell origin, based on characteristics such as the positive expression of S-100 protein determined by immunohistochemical staining. A 29-year-old female was referred from the local clinic because of a polypoid lesion in the gastric cardia. After performing endoscopy and endoscopic ultrasonography, a 1.5 cm submucosal tumor covered with normal mucosa was found in the gastric cardia. Endoscopic mucosal resection revealed a tumor that initiated in the esophagus, and grew into the cardia. Immunohistochemical analysis revealed strong positive staining for S-100 protein, confirming the presence of a granular cell tumor.