BACKGROUND & OBJECTIVES: Congenital heart diseases (CHDs) affect approx 6–8 infants per1000 live births and 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 10years 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 19months and that of controls 18months. 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 1.358.25) and(p<0.001), OR-3.86(95% CI 1.858.11) 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 [OR 4.10(95% C.I=1.34-14.97), co-morbidities [p=0.02, OR2.7 (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.