BACKGROUND & OBJECTIVES:Congenital heart diseases (CHDs) affect approx 6-8 infants per1000 live births have multifactorial origin. Various studies attribute number of maternal (e.g. family history of congenital heart diseases, consanguinity, febrile illness, co-morbidities like diabetes or hypertension) and fetal factors (prematurity, LBW, chromosomal abnormality) for development of CHDs. There is paucity of data in India; hence this study was conducted to evaluate risk factors in causation of CHDs in children. METHOD: It was a case control study conducted from Mar to Aug 2012 among children up to 10 years of age attending tertiary care hospital in Maharashtra, India. A total of 75 cases of CHDs and equal number of matched controls were included in the study. RESULTS: The mean age of cases was 19 months and that of controls 18 months. Male to female ratio was 1.5:1 among cases and 1.7:1 among controls. VSD was the commonest cardiac anomaly found in 37(49.33%) cases. In neonatal characteristics, cases had significantly increased number of prematurity and low birth weight as, compared to control(p=0.006), OR-3.25(95% CI 1. 35-8.25) and(p<0.001), OR-3.86 (95% CI 1.85-8.11) respectively. Twenty six (35%) children among cases were born of consanguineous marriage while 8(11%) in controls and association was found statistically significant [p<0.01, OR-4.44 (95% C.I=1.75-12.24)]. Similar associations were seen with family history of congenital heart diseases ), co-morbidities [p=0.02, OR-2.7 (95% CI 1.1-6.93)]. CONCLUSION: Our study showed maternal factors like consanguinity, family history of congenital heart diseases, co-morbidities like gestational diabetes, hypertension and drug intake during pregnancy were significant underlying risk factors for development of CHDs in children.