Objective: To explore the multiple risk factors for family lifestyle of children with congenital heart defects (CHDs) in Shaanxi Province, China. Methods: A 1:1 case-control study was carried out to investigate 60 pairs of children and their parents. The univariate and multivariable logistic regression models were used to analyze the influence of risk factors related to parents' lifestyle on CHDs. Results: Several possible risk factors were found for CHDs, including fever (OR = 4.465, P = 0.017), pesticides contact (OR = 2.234, P = 0.083), passive smoking during pregnancy (OR = 20.529, P = 0.007) and father's smoking (OR = 3. 342, P = 0.005); fever (OR = 2.428, P = 0.012) and passive smoking during pregnancy (OR = 1.201, P = 0.037) were also correlated with ventricular sepal defect (VSD). Conclusion: Fever, pesticides contact and passive smoking are associated with CHDs during pregnancy. We should focus our attention on health care during pregnancy to avoid the above-mentioned risk factors and call on parents to hold on to a good healthy lifestyle.