Primary EBV-positive mucocutaneous ulcer (EBVMCU) is a rare and indolent disorder occurring in the oropharynx, skin, and gastrointestinal tract, with remission after removal of the immunosuppressive causes. We present a 69-year-old woman with heartburn, regurgitation of gastric acid, enlarged lymph nodes, and parotid glands. The endoscopic examination showed a circumscribed ulcer in the lower esophagus. A biopsy pathology indicated an esophageal EBV-associated lymphoproliferative disorder and a parotid gland/lymph node indolent B-cell lymphoma. Interestingly, the patient did not undergo any treatment, but the endoscopic ulcer improved significantly after more than 2 months. The last pathology showed EBV negativity, and EBVMCU was considered in combination with clinical and endoscopic manifestations. We followed up with the patient at 6 months, and the symptoms of acid reflux and heartburn had disappeared. Our case demonstrates that EBVMCU may occur in the esophagus with spontaneous regression.