Abstract: Objective To make epidemiological investigation on children and teenagers with congenital heart disease (CHD) from October 2015 to November 2016 in three counties in Zhaotong area. Methods The examination was divided into the following two phases: primary screening, secondary screening. Standardized echocardiographic examination was performed for the students with suspected CHD. we combined comprehensive analysis of electrocardiogram, X-ray and cardiac catheterization to make a clear diagnosis for patients. The index of prevalence rate of CHD, the types of the disease, and gender of the patients in 14957 children and teenagers were statistically analyzed. Results Altogether 61 cases of CHD were discovered. The total prevalence of CHD was 4.08‰ (61 of 14957 cases) , the prevalence rate in male was 4.55‰, in female was 3.61‰, No difference of the prevalence rate was found between male and female (χ2=0.823, P>0.05) ;the difference among different age groups had no statistical significance (χ2=4.692, P>0.05) ;The prevalence rate of CHD in Qiaojia was 5.17‰, in Daguan was 6.36‰, and in Zhenxiong was 3.03‰, There was no significant difference in the prevalence rate among different countries (χ2=5.897, P>0.05) . In terms of the types of the disease, atrial septal defect accounted for the first (37.7% ) , followed by ventricular septal defect (26.2% ) and patent ductus arteriosus (19.7% ) , others types of CHD were rare. Conclusion The prevalence of CHD in investigated area is slightly higher than the average level of the nationwide. The main types of CHD are atrial septal defect, ventricular septal defect and patent ductus arteriosus.