Abstract: Objective To analysis the prevalence and tendency of 21 frequently types of birth defects in Guangdong province, and provide basic evidence for prevention of birth defects.Methods The data of 58 birth defects surveillance hospitals in Guangdong was used. 21 types of birth defects which easy to be confirmed diagnose during perinatal period were selected. The mean prevalence was calculated and the total number of each selected birth defect of overall province was estimated..Results The five years mean cumulative prevalence of selected 21 birth defects was 104. 52 per 10 000 births (13 257/1 268 424), and 68. 01% (9016/13 257) of them were live birth. The estimated total number of selected 21 birth defects was 96 75895% CI (96 447, 96 758) from 2012 to 2016 in Guangdong, and the annual total prevalence was 106. 56, 98. 70, 105. 50, 105. 83 and 105. 76 per 10 000 births, respectively. The tendency was stable (χ²=0. 795, P=0. 373). The specific prevalence of each birth defect varies greatly. The prevalence of of cleft palate, hypospadia, ankylodactyly, hyperdactylia, and equinovarus showed an increasing tendency. The prevalence of anencephaly, cleft spine, cenencephalocele, congenital hydrocephalus, cleft lip, cleft lip with plate, limbs crispation, acromphalus, and abdominal cleft showed a decreasing tendency.Conclusion In Guangdong, there is a heavy burden of birth defects, and the interventions on birth defects shows initial effects.