Despite the progress in diagnostic modalities and therapeutic strategies, Infective endocarditis remains a lethal disease especially in children. We aimed to update current characteristics of infective endocarditis in children and to identify complications. We carried out in our department a retrospective study including 40 children diagnosed with infective endocarditis (IE) between 1983 and 2017. Forty children with IE were diagnosed by using modified Ducke criteria. There were 19 boys and 21 girls aged between 1 to 18 years. Sixteen children (40%) had congenital heart disease including inter ventricular septal defect, tetralogy of Fallot, patent ductus arteriosis. Left-side IE occurred in 33 patients (82.5%). There was no case of IE of prosthetic valve. Streptococcus viridans were identified in 4 patients and Staphylococcus aureus in 5 patients. Blood culture-negative IE was concluded in the other cases. Neurological complications occurred in 9 patients, including 5 with cerebral infarction and 3 with intra cerebral bleeding. Clinical signs of cerebral stroke were hemiparesis (63%), facial paralysis (18%) and speech disorder (27%). One case of pulmonary embolism was identified and 3 cases of splenic infraction. The mortality in children with IE was important (50%). The mortality rate in patients who had cerebral stroke was significantly higher than those who had not (75% versus 35%, P < 0.05). In our study, IE in child has various characteristics compared to IE in adult. IE complication increases the risk of death.