Granular cell tumors of the esophagus are rare neoplasms and their diagnosis is mainly based on histopathologic examination of endoscopic biopsies. With the development of endoscopy and iodine staining, there has been a marked increase in local treatment alternatives for early esophageal neoplasms. In this case report, we describe a granular cell tumor of the esophagus in an adult patient and briefly discuss the literature concerning clinicopathologic aspects and management of these uncommon tumors. In this patient, we describe the removal of the tumor by endoscopic submucosal dissection technique.