A 6-day-old neonate with tricuspid atresia and transposition of the great vessels developed acute bacterial endocarditis and the "scalded skin syndrome." A nonphage typable Staphylococcus aureus was recovered from multiple sites prior to death, and staphylococci were also cultured from vegetations on the endocardial surface of the heart at necropsy. This case represents one of the few documented cases of bacterial endocarditis in the neonatal period and is one of the first associated with congenital heart disease. It is also the first recorded case of the scalded skin syndrome associated with bacterial endocarditis.