Objective To assess disease spectrum and clinico-pathological characteristics of infective endocarditis(IE) in patients with congenital heart disease(CHD). Methods Clinical characteristics of IE patients with CHD were investigated,who were receiving surgical treatment and pathological diagnosis during 2010.01-2013.12 in Changhai hospital. The clinical datas，disease species and clinico-pathological characteristics were retrospectively analyzed. Results During these 4 years, there were 58 IE cases associated with CHD whose mean age is (28.3±7.3)years accounting for 33.7％(58/172) of all patients with IE receiving surgical treatment. The top three types of congenital heart disease were included bicuspid aortic valve accounted for 34.5% (20/58), ventricular septal defect(VSD) accounted for 20.7% (12/58) and patent ductus arteriosus(PDA) accounted for 15.5% (9/58). Other congenital heart diseases (including aortic stenosis, subaortic stenosis, aortic sinus aneurysm right ventricular fistula, mitral stenosis, mitral incompetence and pulmonary stenosis) accounted for 13.8% (8/58),while complex CHD (containing two or more abnormalities) accounted for 15.5% (9/58). Mean age of bicuspid aortic valve patient was higher than other CHD patients with infective endocarditis [(38.7 ± 11.7) years vs (26.6 ± 12.4) years, P <0.05]. Positive rate of blood culture were 42.1% (25/58),in which viridans accounted for 48.0%(12/25). Echocardiography was performed in all patients and the positive rate was 96.6%(56/58) It was common that excrescence attached multiple sites, For patients with bicuspid aortic valve, excrescence often attached aortic valve, for VSD patients, excrescence often attached tricuspid septal leaflet and VSD right ventricular surface,while for PDA patients,was aortic valve,opening of PDA or the left pulmonary artery wall eroded by shunt. Conclusion CHD is common underlying disease for IE , in which bicuspid aortic valve ranks first, VSD and PDA also occupy a high proportion. Blood culture positive rate is low, while transthoracic echocardiography(TTE) is recommended as a reliable inspection techniques. Different diseases have different parts of the vegetation involving, more common is that multiple sites are involved.