Background: Infective endocarditis (IE) is a rare disease yet with numerous health hazards. Currently no data is available about IE in Egyptian children. This work aimed to study the epidemiological pattern, mode of presentation, microbiology, and the outcome of IE at Cairo University Children's Hospitals. Patients and methods: A cross-sectional study was conducted over a period of 6 months and included 100 patients diagnosed with IE. All patients were subjected to 1) Full medical history and examination to study their demographic date, detect risk factors, pattern of presentations, and type of treatment. 2) Laboratory investigations including blood cultures. 3) Complete echocardiography, and 4) Close follow up of the patients to determine the outcomes and fate of IE. Results: The age of included patients ranged from 1-168 months, with a mean 59.37 (SD 41.91). They were 63 (63%) males and 37 (37%) females. Most of patients (68%) were from rural areas, and 32% were from urban areas. Most important risk factors are type of congenital heart disease (CHD) (75%), central venous pressure (CVP) insertion (29%), and dental problems (24%). Vegetations were found in 77% of our patients, significantly in the neonatal intensive care units (NICU) (p=0.02). IE was caused mainly by gram positive organisms (85%). Conclusion CHD, CVP, dental problems were the most frequent risk factors of IE in our patients. CHD was associated with significantly prolonged hospital stay and delayed response to treatment. Early and prompt management of CHD is highly recommended to reduce impact of IE in pediatric patients.