Congenital heart disease (CHD) is a chronic illness that increases the risk of infective endocarditis (IE). As a result of improvements in pediatric care, the number of adults with CHD has now surpassed that of children. Researchers wanted to assess the cumulative incidence of IE in CHD patients and discover temporal variations are comparable to controls. Patients with CHD born between 1930 and 2017 were matched with 10 random controls in a nationwide registry-based case-control study. The Swedish 10-digit personal identification number was used to link infective endocarditis occurrences. A total of 89,541 CHD patients and 890 470 matched controls were enrolled in the study. There were 1477 IE episodes in CHD patients and 447 episodes in controls. At the age of 87, patients with CHD had an 8.5% cumulative incidence of IE, compared to 0.7% in matched controls. In CHD patients and controls, the incidence rate of IE per 100 000 person-years was 65.5 (95% confidence interval [CI] 62.2–68.9) and 1.8 (95% CI: 1.7–2.0), respectively. Patients with CHD had an IE incidence similar to that of 81-year-old controls by the age of 18. The prevalence of IE varied by age but not by year of birth. Bacterial etiology was found in half of the IE episodes starting in 1997; streptococci caused 43.3% of CHD IE cases, and Staphylococcus aureus caused 29.8%. Infectious endocarditis is still a significant problem in CHD patients. The number of IE events is projected to rise as the CHD population becomes older because the incidence correlates with age.