Benign tracheal tumors are less common than malignant tumors, but they are relatively more important because they are operable and curable. Neurilemmoma is originated from Schwann cells of nerve sheath, which are characterized by benign, solitary, and encapsulated mass. Tracheal neurilemmomas are extremely rare and reported only 21 cases in the world literature. Recently, we experienced a case of neurilemmoma which arose from the trachea of a 48-year-old female patient who complained of progressive dyspnea. The tumor mass was removed successfully through bronchoscopic and tracheal fissure approach. The final pathological diagnosis viewed under a microscope after H&E stain was a neurilemmoma in which Antoni type A and type B both existed.