The population-based incidence, diagnostic procedures, therapy and survival of thymic epithelial tumours were determined using the Netherlands National Pathological Archives and the Netherlands Cancer Registry. Excess mortality compared to the Netherlands standard population was estimated by relative survival analysis. Between 1994 and 2003, 537 thymic epithelial tumours were diagnosed. The incidence of all thymic epithelial tumours was 3.2/1,000,000. Diagnosis was obtained by primary resection in 56% of cases. Survival data were available for 232 cases. Not only thymic carcinomas (type C) but also thymomas (types B1-B3) were associated with excess mortality. Cases that underwent resection (78%) had a better survival than non-operated cases (median survival >10 years versus 1.1 years, p<0.001). Amongst the surgically treated cases (n=180), the completeness of resection did not predict survival (p=0.53). Thymic epithelial tumours are rare. Excess mortality was observed in the majority of tumours. Surgery offers the best perspectives, even if the resection is incomplete.