Objective To investigate the association between endothelial NO synthase (eNOS) gene G10T polymorphism nd the susceptibility of sporadic congenital heart disease (CHD). Methods The genotype on eNOS G10T locus was detected nd compared in 1323 children with sporadic CHD and 1323 non-CHD children. Results Compared with the CC genotype, the A genotype significantly increased the risk of CHD (adjusted OR=1.42, 95% CI=1.01-2.04). Compared with the CC/AC genotype, he AA genotype significantly increased the risk of CHD (adjusted OR=1.39, 95% CI=1.08-1.92). Based on stratified analysis, he AA genotype was associated with the susceptibility of perimembranous ventricular septal defects (adjusted OR=1.56, 95% CI=1.17-2.47). Conclusions In Chinese population, the eNOS G10T polymorphism may increase the susceptibility of sporadic CHD.