Twenty-three cases of IE, of which 91% had underlying congenital heart diseases were diagnosed by positive blood culture or vegetative lesion detected by 2-D ECHO when a patient revealed clinically suspicious episodes. VSD and TOF were the most common underlying heart diseases. The vegetation was detected successfully by 2-D ECHO in 91% of the cases. The causative organisms were identified in 83% of the cases. Streptococcus viridans and Staphylococcus aureus were the two major organisms detected. Candida albicans was found in 3 postoperative and fatal cases. Embolic complications occurred in 7 cases (30.5%): five cases of pulmonary infarction and two of cerebral infarction. The overall mortality was 22%. We recommend early surgical intervention whenever a patient reveals a very large vegetation with animated movability, and emphasize the importance of a proper prophylaxic regimen.