Benign tumors of the trachea are rare and are usually misdiagnosed as bronchial asthma because of the similarity of the symptoms and signs. Although the prognosis of neurofibroma which originats from Schwann cells is good, it may recur or undergo malignant change, so segmental resection of the trachea is recommended. Recently, we experienced a case of primary neurofibroma of the trachea treated successfully by segmental resection of the trachea and end-to-end anastomosis. We report it with a brief review of literatures.