We report an extremely rare case of primary glomus tumor of the esophagus, and review the corresponding literature. A 66-year-old female underwent a follow-up upper-gastrointestinal endoscopy due to dysphagia and previous history of esophageal lump. Endoscopic ultrasonography revealed a mass involving the deep mucosa and submucosa (Layers 2 and 3). Endoscopic biopsy revealed a mesenchymal tumor composed of nest of epithelioid cells with associated small blood vessels proliferation. Immunohistochemically, the tumor cells were positive for alpha smooth muscle actin and vimentin, but negative for c-kit and the vascular stroma was highlighted by positivity with CD34. Pathological examination confirmed that the tumor was a glomus tumor of the esophagus. The patient remains healthy without any recurrence to date.