Congenital heart defects (CHD) are the commonest birth defects and is an important cause of morbidity and mortality in newborns. The current study was done aiming at determining spectrum and clinical profile of various congenital heart disease (CHD) and extra cardiac anomalies associated with CHD in neonatal period. An observational study was carried out in the newborn unit in Rajah Muthiah medical college and hospital from November 2014 to August 2016. All newborns with the clinical suspicion of CHD were included in the study and were further evaluated by Echocardiography. Clinical spectrum of various congenital heart diseases was observed. Out of 2189 admissions 42 cases were diagnosed with CHD and included in the study. All the cases belong to Acyanotic heart disease. ASD was the commonest contributing to 54.76%, followed by VSD (23.8%) and PDA (7.14%). Six cases were diagnosed with complex CHD. Most of the cases presented with fast breathing and chest retractions. Conclusion: Children having murmurs should be screened unless thought to be physiological. Early diagnosis, close monitoring and timely intervention in cases of CHD will go a long way in reducing the morbidity and mortality to a large extent.