PURPOSE The purpose of this study was to investigate dental caries, bacterial dental plaque, gingivitis and caries related oral microflora in children with predominantly autosomal recessive Dystrophic Epidermolysis Bullosa (DEB). METHODS Thirty children with DEB from The Great Ormond Street Hospital for Children and 31 control children matched for age, gender and ethnicity were included in the study. RESULTS The main findings were: 1. A significantly greater mean dmft in the DEB children (p < 0.05). 2. A significantly greater mean plaque score for the DEB children for both the primary (p < 0.001) and permanent teeth (p < 0.02) compared with the control children. 3. A significantly greater mean gingivitis score for the DEB children for both the primary (p < 0.002) and permanent teeth (p < 0.0001) compared with the control children. 4. A significantly greater salivary total anaerobic count for the control children compared with the DEB children (p < 0.001). CONCLUSIONS The results reflect the difficulties that children with DEB have with basic oral hygiene procedures combined with slow oral clearance.