Objective To analyze the data of birth defects from 2001 to 2013 in Pingyin,and to provide a basis for making effective interventions to reduce birth defect ratio. Methods The datas of perinatal in Pingyin nearly 14 years were investigated and collected,the birth ratio,categories and relevant factors were analyzed in descriptive statistical methods. Results The perinatal birth defects ratio was 10.8‰,the prenatal diagnosis ratio was 20.7%.The top five birth defects categories were hydrocephalus,hypospadias,deformity of external ears,cleft lip and palate,kidney malformation.The male birth defect ratio was higher than that of female,the multiple births was higher than single birth,the maternal age <20 and >35 were higher than other age groups (P all<0.05),there was no significant difference between urban and rural areas. Conclusions The birth defects is related to gender,maternal age as well as the embryo number.The birth defects ratio in Pingyin is lower than the average of China.The prenatal diagnosis rate should be raised for reducing the birth defects.There is great potential for the development of prenatal diagnosis technologies.