Acute bacterial endocarditis in the absence of underlying heart disease is rare. We report the occurrence of endocarditis caused by group A beta-hemolytic Streptococcus (GABHS), following varicella, in a 5-month-old child without heart disease. In addition to this child, seven other children with endocarditis caused by GABHS have been reported since 1966, six of whom did not have preexisting heart disease. In one of these children, GABHS endocarditis was preceded by varicella. These cases indicate that GABHS is capable of causing endocarditis in the absence of heart disease, and they provide further evidence that varicella is an important risk factor for invasive GABHS infections.