Tracheal glomus tumors are extremely rare. We present herein the case of a 43-year-old man with hoarseness who was found to have a glomus tumor arising from the lower trachea. Bronchoscopy and chest computed tomography revealed a polypoid mass in the membranous portion of the trachea just above the carina. The patient underwent successful tracheal sleeve resection and reconstruction. A pathologic diagnosis of a glomus tumor with clear surgical margins was subsequently confirmed, and the patient is well 20 months postoperatively with an intact anastomosis.