Abstract: Objective To understand the occurrence and the related risk factors of birth defects. Methods Descriptive analysis was conducted on birth detect surveillance in the infants during January 2008 to June 2014. Results A total of 777 cases of birth defect were detected in 73498 infants, and the incidence of birth defect was 1.06%. The 5 most common birth defects were congenital heart disease, multi finger (toe), hypospadias, cleft lip, and palate and deformity of external ear. Compared infants born with no birth defects, male, preterm, low birth weight, twin and multiple births and resident were statistically higher in infants with birth defects (P<0.05). The major risk factors of birth defects were the medication history, spontaneous abortion, gestational diabetes mellitus, and family history. Conclusions The incidence of birth defect can be reduced by providing good health care during pre-marriage and pregnant so as to decrease the occurrence of premature infants, twins and multiple births, and low birth weight as well as improving prenatal diagnosis and intensifying birth defects surveillance.