Esophageal submucosal tumors are less common than other gastrointestinal tract tumors. Leiomyoma is the most common benign esophageal SMT, accounting for more than 70% of these tumors. We report on a case of a 56-year-old woman with a 3-cm diameter midthoracic esophageal submucosal tumor. Magnetic resonance imaging suggested leiomyoma or neurofibroma. Video-assisted thoracoscopic surgery was performed to enucleate the tumor from the esophageal wall by splitting the muscle layers. The postoperative course was uneventful, and the patient was discharged on postoperative day 8. Immunohistochemical staining confirmed the diagnosis of esophageal neurofibroma. Gastrointestinal tract involvement of neurofibromatous lesions is rare and occurs most frequently as a systemic manifestation of von Recklinghausen disease. Cases of localized esophageal neurofibroma with prior or subsequent evidence of generalized neurofibromatosis have rarely been documented. This is a rare case of isolated esophageal neurofibroma without classic systemic manifestations of generalized neurofibromatosis, and it is the first reported case treated by video-assisted thoracoscopic surgery.