Two patients with granular cell tumors of the esophagus are presented. Both were men, 54 and 51 years old, respectively, and without tumor-related complaints. Diagnosis was made by esophagogram and endoscopic biopsy. The two patients were treated with a left thoracotomy and local excision of the tumors. A review of the world literature was carried out and data of general interest were collected. The symptoms and management of esophageal granular cell tumors, as well as histogenesis, are discussed.