Primary tumors of the trachea are rare, accounting for less than 0.2% of respiratory malignancies. The most common primary malignant tracheal tumors are squamous cell carcinoma and adenoid cystic carcinoma. Evaluation of tracheal tumors involves CT, bronchoscopy and biopsy to determine resectability while assessing for metastatic disease. Surgical resection is the primary treatment when possible but unresectable disease may be treated with stents or chemoradiation to relieve obstruction and slow progression. Postoperative radiation is recommended for intermediate or high-grade malignancies due to risk of positive margins.