Glomus tumors are neoplasms arising from modified smooth muscle cells surrounding arteriovenous anastomosis in the dermis and subcutaneous tissues, which are contributing to blood flow regulation and temperature control on the skin surface. Glomus cells are sparse or absent in visceral organs, making extracutaneous presentation of glomus tumors an extremely rare finding. We briefly report histological considerations on glomus tumors of the trachea and sum the multidisciplinary aspects of their staged endoscopic and surgical management using the example of a rare case presentation.