The purpose of this work was to investigate the prevalence of dental caries, bacterial dental plaque, gingivitis, enamel defects and caries- related microflora in children with severe haemophilia. Thirty-eight children with severe haemophilia (factor VIII and IX < 2 U dL(-1)) were recruited from Great Ormond Street Hospital for Children and matched for age, gender and ethnicity with healthy control children from the Eastman Dental Institute. Indices were recorded for decayed, missing, and filled teeth and surfaces in both the deciduous dentition (dmfs/dmft) and the permanent dentition (DMFS/DMFT). The plaque and gingivitis scores and developmental enamel defects were also recorded. The caries-related microflora was sampled and cultured for Streptococcus mutans, and Lactobacilli and Candida species. A significantly greater proportion of children with severe haemophilia were caries-free compared with the controls (36.7% vs. 13.3%; P=0.04). Both the DMFS and DMFT were significantly greater in the controls (3.6 and 2.8, respectively) compared with the haemophilia group, (0.8 and 0.7; P=0.007 and P=0.04). The plaque score for the permanent dentition only was significantly greater for the control children (24.2) compared with the haemophilia group, (10.2; P=0.04). The mean number of colony forming units of S. mutans was significantly greater in the control group compared with the haemophilia group (P=0.05). We conclude that children with severe haemophilia have a significantly lower prevalence of dental caries compared with matched, healthy controls.