The article aims to draw the attention of endoscopists, thoracic surgeons, and oncologists to the complexity of the differential diagnosis of non-epithelial (mesenchymal) esophageal neoplasms and present the new possibilities of flexible endoscopy in the treatment of these patients. This article describes the clinical case of successful endoscopic treatment of a patient with an esophageal granular cell tumor. A granular cell tumor (Abrikosov’s tumor, granular cell myoblastoma) belongs to benign non-epithelial tumors and is exceptionally rarely localized in the esophagus. Such neoplasms in the esophagus, as a rule, do not have characteristic clinical manifestations and are usually detected by chance during endoscopic examination. Modern minimally invasive treatment methods, such as endoscopic mucosal resection and submucosal dissection, are successfully used to treat this pathology.