Primary malignant neoplasms of the trachea are very rare and data relating to them are limited. This study was conducted to review the presentation, management, and outcomes of primary tracheal cancers at our institution, a large multidisciplinary cancer center.Retrospective chart review was conducted for all patients found to have a pathologic diagnosis of primary tracheal malignancy.Since 1945, 74 patients were diagnosed with primary tracheal cancers. Among these, 34 (45.9%) were squamous cell carcinomas, 19 (25.7%) were adenoid cystic carcinomas, and 21 (28.4%) were of other histologic types. Presenting symptoms were most frequently dyspnea (55.4%), hemoptysis (48.6%), cough (41.9%), and hoarseness (35.1%). Most patients (77.3%) were former or current smokers, particularly those with squamous cell carcinoma (93.3%). For the entire group of 74 patients, the 5-year disease-specific mortality rate was 72.9% and the 5-year all-cause mortality rate was 79.3%. Patients who had adenoid cystic carcinoma and those with cervical primaries had better rates of disease-specific and overall survival than others (p = 0.036 and 0.006 for the former patient group and p = 0.006 and 0.030 for the latter patient group). Among patients with incident disease treated at our institution (n = 45), those undergoing primary operation with adjuvant radiotherapy appeared to have better disease-specific and overall survival rates compared with those undergoing primary radiotherapy with or without chemotherapy (p = 0.0002 and 0.0003, respectively). Although those undergoing operation and receiving radiotherapy did better than those undergoing operation alone, the difference was not statistically significant.Primary tracheal cancers are very rare, and our results should be viewed with caution, given that our population comprised a small heterogeneous group treated over a 60-year period. Although squamous cell carcinoma was the most common pathology in smokers, adenoid cystic carcinoma was more prevalent among nonsmokers. Operation with adjuvant postoperative radiotherapy is recommended for most patients.