Abstract: Objective To investigate the birth defects-related factors in order to take countermeasures against them. Methods Data about birth defects in our hospital from 1996 to 2011 were retrospectively analyzed. Results Of the 10 025 perinatal infants, 217(21.65‰) had birth defects. The rate of birth defects was significantly lower in 1996-2003 than in 2004-2011. The birth defects were related with the maternal age, newborn gender, fever in the early gestational period, taking drugs and exposure to toxic or harmful substances. The incidence of congenital heart disease was the highest in perinatal infants with birth defects. B ultrasound plays an important role in prenatal examination. Conclusion The incidence of birth defects should be reduced by improving their prenatal diagnosis, strengthening the good birth and good care awareness and enhancing the population quality.