Two patients with esophageal granular cell tumor (GCT) are presented. Both were women and complained of esophageal symptoms. In one patient the tumor was in the submucosa of the lower esophagus and the correct diagnosis was possible after surgical enucleation through a left thoracotomy. In the other patient a middle esophageal GCT eroded the mucosa and was diagnosed by endoscopic biopsy. The latter patient refused planned surgery and had subsequent clinical and endoscopic controls. Both management modalities at a later follow-up proved effective.