Abstract: Objective To investigate the maternal exposure risk factors during periconceptional period and infants congenital heart disease(CHD) incidence risk. Methods All infants were collected from Xijing Hospital and diagnosed with CHD from March 1st, 2012 to October 1st 2013. Healthy controls were selected from those infants with no CHD after ultrasonic cardiogram at the same time. The questionnaire were carried out among CHD cases and controls including general information of infants and their parents, the maternal exposure to environmental risk factors during periconceptional period. Results 435 infants with congenital heart disease and 574 healthy controls were included in this study. Results showed that medicine application during the first trimester of pregnancy (OR=4.65, 95% CI:2.84-7.66), virus infection(OR=3.15, 95% CI:1.66-6.01), home decoration(OR=1.87, 95% CI:1.24-2.80), hair perming and dying(OR=2.18, 95% CI:1.37-3.49)and parents work environment exposures during peri-conceptional(OR=2.88,95% CI:1.86-4.47) are all independent risk factors for congenital heart disease. The risk of CHD increased significantly as the number of risk factors increased. Compared with 1 risk factor, exposure to 3 or more risk factors leads to a 16-fold risk of CHD. Conclusions There are significant association between risk factors exposure during periconceptional period and CHD in infants.