Abstract: Objective To further understand the incidence of birth defects in Xiangshan and facilitate the implementation of birth defect intervention project. Methods The analysis was conducted on the surveillance data off birth defect detected during pregnancy and 7 days after birth from 1 January 2005 to 31 December 2009 in Xiangshan. Results Totally 24 863 infants were born during this period, the incidence of birth defect was 147.61/lakh; 170 dead fetuses were detected, 65.88% of which were caused by fetus defect. The fetus defect detected before and after 28 weeks of gestation accounted for 11.99% and 88.01% respectively. The first 5 birth defects were congenital deformity in circulatory system, congenital deformity in musculoskeletal system, congenital deformity of eye, ear, face and neck, congenital deformity in nervous system as well as cleft lip and cleft palate. Conclusion The overall incidence of birth defect increased in Xiangshan during this period. The incidence of fetus defect in dead fetuses was significantly higher than birth defect in live fetuses. The proportion of fetus defect detected before 28 gestation weeks increased by years. It is essential to conduct tertiary prevention of birth defect.