The esophagus is the most common site of origin of gastrointestinal tract granular cell tumors. Approximately 270 cases of esophageal granular cell tumors have been reported in the literature. Most esophageal granular cell tumors are found incidentally during endoscopy. Although granular cell tumor of the esophagus has become easily recognizable by its endoscopic features, it has to be differentiated from other benign and malignant mucosal and submucosal lesions. The majority of esophageal granular cell tumors are asymptomatic and benign; thus, close follow-up of the patients with endoscopy could be considered sufficient as a therapeutic management. New therapeutic options should be considered especially for larger lesions. Three cases of granular cell tumors with complaints of epigastric discomfort, regurgitation, nausea, and vomiting, which were detected in the lower part of the esophagus on upper gastrointestinal tract endoscopy, are discussed with the most recent literature review on this subject.