Sixty-two cases of endocarditis occurring in children between January 1977 and February 1992 were reviewed and compared with series from the 1970s and early 1980s. Changes in risk factors, pathogens, diagnostic modalities, and outcome were determined. Complex congenital heart disease (22 cases) and unrepaired ventricular septal defect (9 cases) were the most common underlying lesions. A total of 19 children with normal anatomy had endocarditis; 6 had community-acquired infection and 13 had hospital-acquired endocarditis (11 of these 13 children had central venous catheters in place, including 7 premature infants). Echocardiograms revealed vegetations in 25 of 49 patients; 24 of these patients had positive echocardiographic findings on the first study. Echocardiographic findings were most often negative in children with complex cyanotic heart disease. Staphylococcus aureus (39%) was the most common pathogen isolated and was associated with a higher incidence of central nervous system complications (p < 0.0015) and a greater need for surgical intervention (p = 0.01) than were other pathogens. Methicillin-resistant S. aureus (eight cases) and coagulase-negative staphylococci (three cases) emerged as important pathogens but were not associated with increased morbidity or mortality rates. Fungal endocarditis (six cases) had a 67% mortality rate. Overall the mortality rate was 11%. Endocarditis remained undiagnosed in seven seriously ill patients until postmortem examination. This study indicates that, during the past decade, important changes in risk factors, pathogens, and the susceptible population have altered the presentation and management of endocarditis in children.