Introduction: The work to be performed on the child Down Syndrome (DS) with Congenital Heart Disease (CHD) must be carried out by qualified professionals and provide these conditions to expand their capacities, helping them to achieve independence in the activities of self-care. Objective: To delineate the independence profile in the self-care activities of DS children who present CHD in the age group of 3 to 7 years and 6 months old and who receive outpatient follow-up at a cardiology reference unit in the city of Belém, Pará, Brazil. Method: This is a quantitative study, in which 18 children, male and female, were followed up at the reference outpatient clinic in cardiology in the state of Pará. To collect data, a personal information interview was conducted with the parents/caregiver and part I of the Pediatric Invalidity Assessment Inventory (PEDI) regarding the child's abilities for self-care activities. Results: The study showed that children with DS and CHD presented impairment in the area of fine motor skills, which interferes with activities such as handling of locks, opening, and closing of faucets, use of utensils, among others. Conclusion: Both DS and CHD are conditions that interfere in the development of skills necessary for independence in self-care activities. Thus, the study demonstrated that the functional performance in the self-care activities of children studied is lower than in children with typical development.