Glomus tumor of the trachea is extremely rare. There were approximately 15 reported cases before. Herein, we report another case of glomus tumor of the trachea in a 50-year-old woman presenting with cough and dyspnea for 8 years. She suffered from hemoptysis for 1 day before this admission. Bronchoscopy and CT scan showed a polypoid tumor protruding into the tracheal lumen and with extraluminal extension. The tumor was located at 9 cm below the vocal cord and 1.5 cm above the carina. It measured 2.5 x 2.5 x 2.0 cm and arose from the posterior wall of the trachea. Microscopically, the tumor consisted of a sheet of uniform cells surrounding the vascular spaces. Only few scattered tumor cells showed weak positive staining for muscle actin (HHF-35) by immunohistochemical stain. Ultrastructural study confirmed the presence of small amount of myofibrillar bundles with focal densities in some of the tumor cells. Other cells exhibited only rare or very sparse myofilaments. Characteristic feature of fine pinocytotic vesicles along the plasma membrance of the tumor cells was also noted.