Introduction: Glomus tumors (GTs) of the trachea are a rare soft tissue disease, originating from modified smooth muscle cells. Patients are usually presented with nonspecific symptoms such as cough, dyspnea or hemoptysis. Surgical treatment has been reported, including thoracotomy and bronchoscopic electro-cautery, there is no doubt that surgery is the treatment of choice, however few studies have evaluated the ideal treatment for tracheal GTs. Case Report: We describe a case of glomus tumor originated at large airway, a 62-year-old woman who presented with cough, and expectoration for 7 days, had been diagnosed as having a tracheal tumor by thoracic computed tomography. Tumor resection was attempted by fibe-roptic bronchoscopy but there were positive edges, therefore surgical tracheal resection and anastomosis were performed. The patient achieved good results and no recurrence was observed during a 10-month follow-up. This is the third report of glomus tumor of the tracheal originated at large airway (trachea and right main bronchus).