A neurofibroma originated from the intrathoracic vagal nerve was presented. A 26-year-old male was found to have an abnormal shadow in the right chest filed on a routine examination. He was complicated with Von Recklinghausen's disease and showed multiple "cafe au lait" spots over his extremities and trunk but no subcutaneous mass. A block resection of the mass was carried for the mediastinal tumor. The tumor was arising from the right intrathoracic vagal nerve which located just below the origin of recurrent nerve and was measured 3 X 3 X 6 cm. Histological examination of the specimen revealed neurofibroma. His postoperative course was uneventful. A brief review in Japan was made on 22 cases collected from the literature.