Objective: The aim of this study was to determine developmental enamel defects and dental treatment conditions in children with congenital heart disease by comparing them with a control group of healthy children. Methods: Children included in the study were referred to a paediatric dentistry for dental examination and treatment after undergoing routine examination in a paediatric clinic. Results: The congenital heart disease group included 72 children and the control group included 56 healthy children. Children in the age group of 3- 14 years were included in this study. The mean age of the congenital heart disease group and control group was 6.24 +/- 2.85 and 6.73 +/- 3.01, respectively. The mean values of the decayed, missing, and filled indices for primary and permanent teeth in the congenital heart disease group were 2.80 +/- 3.77 and 0.81 +/- 1.63, respectively. In the control group, the values were 1.87 +/- 3.31 and 0.72 +/- 1.46, respectively. The care score for primary teeth was 3.6% in the congenital heart disease group and 13.3% in the control group. The enamel defect was detected in at least one permanent tooth in seven out of 72 children (9.7%) in the congenital heart disease group and in three out of 56 children (5.3%) in the control group. Conclusion: Although there was no significant difference in the development of dental caries or the prevalence of enamel defects between children with congenital heart disease and healthy children, the care score was low in children with congenital heart disease. In addition, children with congenital heart disease had a higher rate of pulled primary teeth and delayed treatment of decayed teeth.