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Abstract Intrathoracic neurofibromas originating from the vagus nerve in patients without von Recklinghausen disease is rare and poses a problem in etiological diagnosis. Surgical resection is usually necessary for precise diagnosis of such tumors. We report the first case of a neurofibroma originating from the right pulmonary branch of the vagus nerve in a 34-year-old male without von Recklinghausen disease. The diagnosis was suggested by the radiological features and was confirmed histologically after resection.