Primary tracheal cancer is considered to be relatively rare. Its epidemiology, therapeutic strategy and prognosis are not well understood. We retrospectively investigated the clinicopathological aspects of 20 patients with primary tracheal cancer. Patients included 11 men and nine women with a mean age of 57.3 years. There were 12 squamous cell carcinomas and eight adenoid cystic carcinomas. Four patients received only palliative therapy. Sixteen patients underwent surgical treatment such as segmental tracheal, laryngotracheal, or carinal resection. One patient with squamous cell carcinoma died of postoperative mediastinitis. Although resected specimens from five patients had tumor positive margins, only one of those patients died after local recurrence and only three patients had postoperative treatment. The 5-year survival rate for patients who underwent surgery was 72.3%. Surgical treatment is the first choice therapeutic modality for primary tracheal cancer in consideration of its prognosis. While performing the operation, safety of the anastomosis should take precedence over completeness of resection.