Objective To investigate one year old babies with congenital heart disease (CHD) during 2009 to 2011 in Haining, and to provide a reference for preventing and reducing the incidence of CHD. Method After medical examination for live birth babies with full 28 weeks of pregnancy, the suspected ones were suggested to take echocardiography (FECHO) to diagnose the CHD. Results A total of 13 593 live birth babies were monitored during 2009 to 2011, and 108 of them were diagnosed as CHD, which made the incidence rate 0.79%.The top three simple CHD types were turned out to be ventricular septal defect (27.78%), atrial septal defect (25.00%), and patent ductus arteriosus (12.04%).The incidence rate for babies diagnosed as CHD was 3.96% (19/480) with gestational age shorter than 37 weeks; meanwhile, the incidence rate was 0.68%(89/13 113)for babies whose gestational ages were within the range of 37 to 42 weeks, which showed demonstrates significant gap (χ²=63.19, P＜0.01).CHD incidence rate for low birth weight children was significantly higher than normal and huge weight children (χ²=123.48, P＜0.01).CHD incidence rate for cases that mothers' maternal age no less than 35 years old was significantly higher than the other two groups (χ²=28.89, P<0.01). Conclusions Premature birth, low birth weight, old maternal age have a certain relationships with the occurence of CHD, which might be the reason leading to CHD for one year old babies in Haining city.Publicity for progestation awareness and prenatal care as well as eliminating negative factors are all demanding to prevent and reduce the CHD incidence rate, thereby improving the quality of births in Haining city.