Objective: to characterize the clinical, dental and epidemiological profile of children with congenital heart disease followed at the dental outpatient clinic of a Maternal and Child University Hospital in the city of São Luís/MA, Brazil. Methods: The present study had a descriptive, retrospective and quantitative nature. Data collection was carried out by analyzing the medical records of patients seen from January 2017 to December 2019, aged 0 to 12 years and of both sexes. Results: The majority of patients were male (51.4%), from the city of São Luís and metropolitan region (52.9%), self-declared mixed race (62.9%). Acyanotic congenital heart diseases were the most prevalent (81.4%) and half of the patients (50%) had already had an episode of toothache. The use of fluoride toothpaste and toothbrush was observed in almost all patients (90%), with the highest frequency of tooth brushing being twice a day (44.3%). Caries disease was the most prevalent oral clinical manifestation (61.4%). Conclusion: The importance of maintaining an adequate standard of oral health and its influence on the general systemic condition of these patients is reaffirmed. It is suggested that more studies be carried out in order to deepen knowledge about this population.