Aim of study: Evaluating the oral health status of children with congenital heart disease in comparison with the healthy children in Lattakia city. Materials and Methods: A total of 100 children aged between 5 and 12 years attending the pediatric clinic of AL-Assad Hospital-Lattakia were included. The study group was consisted of 50 children diagnosed with heart disease. The control group was consisted of 50 healthy children of the same age and gender. Dental caries, plaque, gingivitis and developmental enamel defects were assessed for each child in the two groups. Results: Mean dmft, gingivitis and plaque were significantly higher in the cardiac group as compared to the control group. Statistically, no significant differences were found in the comparison between the prevalence of developmental enamel defects and mean DMFT for the two groups. Conclusion: Children with congenital heart disease had a poor oral health compared to the healthy children, which increased the risk of susceptibility to bacteremia and development of infective endocarditis. Therefore, dental care for children with heart disease must be seriously considered at an early age when the first tooth erupts.