Background. Adenocarcinoma of the ethmoid sinus is rare. EUROCARE data provide a good opportunity to study the survival of this rare disease in a population of continental size. Patients and methods. A total of 204 cases, age 15 to 99 years, diagnosed with primary ethmoid sinus adenocarcinoma between 1983 and 1994, were analyzed. The data were contributed by 22 population-based cancer registries from the nine countries participating in EUROCARE. Relative survival by sex, age, period of diagnosis, region and stage, and adjusted relative excess risk (RER) of death, were estimated. Results. Survival was 83%, 58% and 46%, 1, 3 and 5 years, respectively after diagnosis. Five-year survival was best (60%) in patients of 55-64 years and worst (33%) in the oldest age group (>/=65 years). Five-year survival differ between European population: in Norway (55%, 95% confidence interval 26.4-80.9) and western Europe that includes populations from Eindhoven, Saarland, Geneva, Italy and France (56%, 95%CI 41.3-68.9) was higher than in the UK (41%, 95% CI 30.8-51.8) and eastern Europe which includes Slovakia and Slovenia, (22%, 95% CI 3.5-54.4). Five-year survival did not improve over time. Due to the rarity of the disease, all the survival differences did not reach the statistical significance. Conclusions. Since no survival improvement with time was evident from this study, efforts should be made to improve early diagnosis. GPs and ENT specialists should be alerted to the disease and encouraged to take occupational histories in people with persistent nasal symptoms, which may lead to a reasonable suspicion of malignancy. Monitoring of exposed workers may also improve early diagnosis. Patients with suspected ethmoid cancer should be referred immediately a specialized diagnosis and treatment centre.