Abstract Primary tracheal tumors are rare neoplasms which often present with indolent and nonspecific symptoms. Despite a tendency for late presentation, more than half of patients with benign and malignant neoplasms will be candidates for curative resection upon diagnosis. This review describes the diagnostic and staging evaluation of tracheal neoplasms. Curative surgical procedures including laryngotracheal resection, tracheal resection, and carinal resection are discussed. Palliative therapies such as external beam radiation, endolumenal brachytherapy, bronchoscopic laser resection, and endoprosthetic stenting are reviewed.