RESUMO
A 29-year-old male with known von Recklinghausen's disease is presented. The main symptom of the patient was paroxysmal episodes of pain and numbness in the right upper hand for the last 10 years. Cervical and mediastial magnetic resonance imaging (MRI) revealed 3 large tumors originating from the right vagus nerve and another of the same origin contralaterally. Surgical resection of the masses in the right hemithorax was performed via right posterolateral thoracotomy. The postoperative course was uneventful and symptoms recessed. Plexoid neurofibromas were diagnosed at histological examination. The mass in the left hemithorax is under surveillance according to the patient's preference. The clinical, radiological, surgical, and histopathological features of this rare case are discussed.