Congenital heart disease in the newborn is a unique and complex problem faced by both pediatricians and cardiologists as it requires skillful handling and balancing of both neonatal issues as well as cardiac physiology. Babies with cyanotic and acyanotic heart disease are physiologically different from each other and management has to be tailored to the individual diagnosis and clinical status. Rapid diagnosis and appropriate management is the key to reducing mortality and morbidity in this fragile patient population. The following review deals with the common presentations of heart problems in a newborn baby and methods of diagnosis, investigative modalities, recent advances and approach to management.