Neurllemomas of the tracheobroncheal tree are extremely rare. Most are located in the lower trachea, and cause chronic cough and wheezing. They usually have a very long natural history, causing symptoms only after they have attained a considerable size. Current treatment of primary intratracheal tumor is sugical removal. Recently, we experienced a case of primary intratracheal neurilemoma which was successfully treated by tracheal resection and anastomosis. We report this case with a brief review of literature.