Granular cell tumors (GCT) of the esophagus are stromal lesions originating from the Schwann cells of the submucosal neuronal plexus. Although they are very infrequent, they constitute the second largest cause of non-epithelial tumors in the esophagus after leiomyomas. These tumors are generally benign, although a certain number of malignant, aggressive cases have been reported. Diagnosis requires that this possibility be ruled out before deciding on which course of therapeutic action to take as well as familiarization with the relevant indicators. GCT linked synchronically or metachronically to other malignant neoplasias of the esophagus have also been described, but the actual extent of this association is uncertain. This report describes five cases of GCT recently diagnosed as incidental findings following endoscopic exploration. All of these were benign and were treated conservatively. The article discusses new aspects relating to the diagnosis of these lesions and the role carried out by endoscopic ultrasonography in their characterization, both at preliminary diagnosis and monitoring levels. No standard therapeutic guidelines exist for the management of GCT, but endoscopic treatment without invading the muscularis propria layer would be used for symptomatic patients, creating histopathological doubts requiring research on the entire organ. Endoscopic therapeutic techniques are analyzed (resection with forceps or diathermy handles, yttrium-aluminum-garnet laser ablation, alcohol injection) in esophageal GCT, which have overtaken surgery in most cases due to their efficiency, greater safety and fewer complications.