Primary tracheal cancer (PTC) is rare. Although histological components of the trachea are similar to that of the lung, the reason for its low incidence is still unknown. Because of its rarity, there are no standard treatment strategies. PTC is not clearly defined. We defined it as a primary malignant neoplasm arising from the sub vocal cord region to the bilateral main bronchi via the bifurcation. The clinical features and prognosis of patients with PTC from 1999 to 2018 at the Kanagawa Cardiovascular and Respiratory Center were analyzed. A total of nine cases, 4 males and 5 females with the median age of 62 years (range:29-81), were included in this study. The clinical symptoms were 3 patients with a cough, 2 with expiratory noise, 2 with hemoptysis, 1 with wheezing, and 1 PTC was detected on screening computed tomography. The tumors were located at upper trachea region in 4 cases, at the lower in 4 cases, and at main right bronchus in 1 case (upper tracheal region is defined as the sub vocal cord area to the lower end of the thyroid and lower tracheal region is defined as the lower end of thyroid to the bifurcation). The histopathological types were 5 (55.6%) adenoid cystic carcinomas (ACC), 2 (22.2%) mucoepidermoid carcinomas, and 2 (22.2%) squamous cell carcinomas. Five patients (55.6%) received radical surgeries and 2 patients (22.2%) who had distant metastasis received palliative surgeries. The remaining 2 (22.2%) patients received tracheal stenting. The postoperative adjuvant therapies included 2 cases with irradiation, and 1 case with chemotherapy. The 2 tracheal stenting cases received full dose irradiation therapy (including case with concurrent chemotherapy) and the 2 cases with palliative surgery also received adjuvant irradiation and chemotherapy, respectively. The overall survival rate was 55.6% at 5 years in all cases. The ACC group tended to have a better prognosis compared to that of the non-ACC group, having the 5-year survival rate was 80.0% vs 25.0%, respectively. In the ACC group, only 1 case (20.0%) died from ACC. The overall survival rate of the radical surgery group and the non-radical group was 60.0% vs 50.0% at 5 years, respectively. We found that PTC generally had a poor prognosis. Among the different types of PTC, ACC had a better prognosis than the other histopathological types. Complete resection achieved a better prognosis, especially in ACC.