Background: In a vast country like India, equitable child health, early detection and treatment of the childhood problems is of paramount importance for ensuring a healthy and dynamic future. The proportion of perinatal deaths due to congenital malformations is increasing as a result of reduction of mortality due to other causes. Intrauterine diagnosis and fetal therapy has become a burning topic nowadays. Present study was conducted to determine the spectrum of congenital anomalies is our institute. Aims and objectives: The study was carried out with the objectives to find out the incidence and patterns of congenital anomalies and to evaluate any maternal and environmental risk factor. Materials and Methods: Hospital based observational study conducted over a period of 2 years (October 2015 to October 2017) in pediatrics and O & G dept. of SCB Medical College, Cuttack, India. The selected newborns were examined in detail and also investigations were done for the presence of congenital anomalies. Various data obtained were displayed in tables and charts. Statistical analysis of the observations were done using percentages, mean, S.D, P-value and chi-square test. Results: Out of 1305 cases, 39 were found to have some form of congenital anomaly with an incidence of 2.98%. It was related to musculoskeletal system (33.3%) mostly followed by gastro-intestinal (23.1%). In our study males (71.8%) showed more anomalies than female babies. Mean age of the mothers was 20-30 years. Parents belonging to low socio-economic status had more chance of babies with anomaly (61.5%) Conclusion: Proper prenatal counseling and education is required for the females. Avoidance of teratogens will help to some extent. Proper nutrition and care of pregnant women should be taken to prevent prematurity and LBW babies. Antenatal visits should be proper, screening and interventions should be early, diagnosis and surgical correction of the malformed babies offer the best chance of survival.