Neurilemmomas of the trachea are extremely rare. The most common site of them is the distal third of the trachea and the age of the patients at presentation varied from 6 to 78 years old. They usually have a freqeuntly very long natural history, causing symptoms only after they have attained a considerable size. We experienced a case of near-total obstruction of the trachea by an intraluminal sessile neurilemmoma. The patient was a 66-year-old man with 2-year history of progressive exertional dyspnea and had several episodes of pneumonitis associated with productive cough. Grossly, the tumor was a well-circumscribed mass. Microscopically, typical cellular Antoni A and myxoid Antoni B areas were revealed.