【Objective】 To investigate the incidence and epidemiologic characteristics of birth defects in Shaanxi province,then to provide scientific evidences for the key of future work. 【Methods】 All perinatal infants aged from 28 weeks of gestation to 7 days after birth in 25 monitoring hospitals in Shaanxi province from 2006 to 2010 were monitored for birth defects.The data were entered by Excel 2003 and analyzed by SPSS 13.0 software. 【Results】 The incidence of birth defects in Shaanxi province from 2006 to 2010 was 117.8 per 10 000.There was an annual ascending tendency in incidence of birth defects(χ²=16.81,P<0.01).The first five main birth defects were total cleft lip,neural tube defects,congenital heart diseases,polydactyly,congenital hydrocephalus,respectively.The incidence of congenital heart diseases showed a year by year ascending trend(χ²=8.58,P<0.01).The incidence of birth defects in rural areas was significantly higher than that in urban area(χ²=70.72,P<0.01). Single defects took up 83.5%.There were 52.2% cases diagnosed by ultrasound and 45.9% cases diagnosed by clinical.The Prenatal diagnosis rate was 48.1%.The perinatal mortality rate was 8.3‰ and showed a year by year descending trend(χ²=6.91,P<0.01).During the five years,the mortality among defective perinatal infants was significantly higher than that among non-defective perinatal infants,and the incidence of birth defects among dead perinatal infants was also significantly higher than the incidence of other cause of death. 【Conclusions】 The incidence of birth defect in Shaanxi province from 2006 to 2010 is lower than the nationwide contemporary level.Much attention should be paid to reduce the incidence of birth defects in rural areas, carry out the preventive work of neural tube defect and improve the prenatal diagnosis ability of all monitoring hospitals.