We report a case of pediatric pneumococcal endocarditis (PPE) and review the English language literature on this disease. Thirty-two cases of PPE were identified since 1900. One-fourth of these were reported since 1990. Clinical features differed from adult cases, with mitral valve involvement being more frequent and Osler’s triad rarely present in children. Congenital heart disease was the only identifiable risk factor. Medical therapy alone resulted in a high mortality rate that was improved in the group of patients receiving combined medical and surgical interventions. PPE is a rare infection that has been reported more frequently in the era of increasing antibiotic resistance. Unlike typical “subacute” endocarditis caused by viridans streptococci, PPE is an aggressive disease with a high mortality rate. Early surgical intervention might improve survival.