Background Tracheal glomus tumors are rare mesenchymal neoplasms that have the potential to cause malignant, central airway obstruction. They require a thoughtful approach to safely secure the airway and definitively resect the tumor. Case Description We report the clinical course of a 25-year-old man in severe respiratory distress secondary to tracheal glomus tumor and the subsequent surgical management. Conclusion Due to their hypervascular nature, greater familiarity with tracheal glomus tumors is needed to ensure appropriate preoperative planning and intervention.