BACKGROUND: The incidence of coeliac disease varies internationally. AIMS: To assess the incidence of childhood coeliac disease in The Netherlands and to study the clinical features and the presence of associated disorders. SUBJECTS: Identified cases of childhood coeliac disease in The Netherlands in 1993-4 by means of the Dutch Paediatric Surveillance Unit. METHODS: Inclusion criteria were born in The Netherlands, diagnosed with at least one biopsy of the small bowel in 1993-4 and age at diagnosis 0-14 years. The data were cross checked by the Dutch Network and National Database of Pathology and compared with data from a previous study on childhood coeliac disease, 1975-90. RESULTS: A total of 193 coeliac patients were identified by means of the Surveillance Unit, another 20 through the National Database of Pathology. The mean crude incidence rate of diagnosed childhood coeliac disease was 0.54/1000 live births, which is in the range of rates found in other western European countries and significantly higher than the mean crude incidence rate of 0.18/1000 live births found in The Netherlands in 1975-90. The clinical presentation was classic: chronic diarrhoea, abdominal distension, and growth failure. Associated disorders were present in 11.7% of the cases. CONCLUSIONS: The incidence of diagnosed childhood coeliac disease in The Netherlands seems to have increased significantly during the past few years. In a period of 20 years no significant changes could be found in the clinical picture at preentation of coeliac disease in Dutch children.