Background: The Congenital anomalies were estimated to be the fifth largest cause of neonatal deaths in India. The purpose of our study was to determine the proportion and pattern of congenital anomalies and their association with risk factors like maternal diabetes, advanced maternal age and consanguinity among the babies born in a tertiary care teaching hospital in Chennai.Methods: This study is a cross sectional descriptive study where all the newborns including still born delivered with the presence of congenital anomalies and those fetuses terminated due to detection of presence of congenital anomalies were analyzed. The study was done at the OBG department in a tertiary care center for a period of one year 1st June 2015 to 31st May 2016.Results: The overall incidence of birth defects were 2.36%, of which musculo skeletal defects (18.88%) were commonly found. There was a definite correlation of risk factors with consanguinity showing an incidence of 1.5 times the overall incidence of birth defects (3.54%) and maternal diabetes mellitus was 6 times the total incidence (15.3%). The correlation between the incidence of birth defects and advanced maternal age was not statistically significant in our study.Conclusions: The study depicts the risk of occurrence of congenital anomalies in the presence of maternal diabetes and in consanguineous marriage and emphasizes on the necessity of adequate screening like first trimester and second trimester ultrasonogram for the early detection of anomalies in the fetus especially if these risk factors are present in the mother.