Abstract: Objective To investigate the incidence, distribution and trend of birth defects in Fengxian District of Shanghai. Methods The population-based surveillance data of birth defects from 28 gestational weeks to 42 days postpartum was retrospectively analyzed. Results A total of 15 744 perineonates were monitored, in which 277 cases of birth defects were detected. From 2008 to 2010, the rate of birth defect was 138.94 per 10 000,162.28 per 10 000 and 230.66 per 10 000, respectively, which indicated a rising trend of incidence. The main types of birth defects in turn were congenital heart disease, deformity of external ear, umbilical hernia, synpolydactyly, syndactyly, chilopalatognathus, hemangioma, hydrocephalus, and neural tube defect. Conclusion The rise of birth defects in recent years should be taken seriously. The surveillance and the comprehensive prevention on birth defects should be strengthened.