BACKGROUND: The aim of this study was to assess the incidence, characteristics, treatment, and survival of patients with tracheal malignancies in the Netherlands. METHODS: All cases of tracheal cancer entered into the database of the Netherlands Cancer Registry in the period 1989-2002 were selected. Data on histological type, age at time of diagnosis, treatment, and survival were analyzed retrospectively. RESULTS: The annual incidence was 0.142 per 100,000 inhabitants (308 cases, of which 15 were found incidentally at autopsy). Of these, 72% were men. In 52.9%, the histological type was squamous cell carcinoma and in only 7.1% adenoid cystic carcinoma (ACC). Mean age at time of diagnosis was 64.3 years. Of the 293 patients diagnosed while alive, 34 patients underwent surgical resection (11.6%), 156 patients received radiotherapy (53.2%), and 103 patients neither (35.4%). Median survival of all 293 patients was 10 months (mean 28 months) with 1-year, 5-year, and 10-year survival rates of 43%, 15%, and 6%, respectively. The prognosis of patients with ACC was significantly better. The 5-year survival rate in patients who underwent surgical resection was 51%, and the 10-year survival rate in these patients was 33%. CONCLUSION: The prognosis of patients with a tracheal malignancy is usually poor. Surgical treatment, however, can lead to good survival rates; still, this is currently only used in selected patients, even though it would seem to be possible in more cases in view of the technical advances in the field of tracheal surgery. Centralizing the care and treatment of tracheal cancers and implementing a more assertive attitude towards this disease could make surgery accessible to a larger number of patients. Data from the literature show that this would lead to better survival in patients with a tracheal malignancy.