We report herein a case of a 46 year old man presenting with a gastric ulcer in whom an endoscopy happened to detect an elevated lesion in the lower esophagus. Endoscopic biopsy proved sufficient for determining the diagnosis of a granular cell tumor (GCT). Electron and microscopic studies suggest that GCT are derived from Schwann cells. Although commonly found in the tongue and skin, GCT are rarely seen in the gastrointestinal tract, especially in the esophagus. However, advances in endoscopic techniques will increase the opportunity of detecting GCT of the esophagus.