We present a 61-year-old man with a glomus tumor of the trachea. To our knowledge, this is the 6th reported case in the literature. The patient was asymptomatic, and bronchoscopic examination revealed a highly vascular polypoid mass about 6.0 cm above the bifurcation of the trachea. We undertook a sleeve resection of the trachea with end to end anastomosis through a right posterolateral thoracotomy. The patient's postoperative course was uneventful. The tumor was 1.2 cm in diameter and was composed of glomus cells, which resembled to modified smooth muscle cells, ultrastructurally.