Congenital anomalies affect approximately 1 in 33 infants leading to 6.6% deaths in infants and causing significant morbidity in children. Ever since the discovery of Penicillin by Ian Fleming a lot of antibiotics have been introduced along with development in immunology and medicine also the understanding of the preventive aspect of the infective diseases has lead to significant reduction in the morbidity and mortality of infectious diseases. But these have little impact on congenital anomalies but with the advancement of antenatal ultrasonography and availability of trained paediatric surgeons the congenital anomalies are being identified and treated well. Hence based on above findings the present study was planned for Assessment of Prevalence of Different Congenital Anomalies in Newborn Admitted in ICU of Nalanda Medical College, Patna, Bihar. The present study was planned in Department of Pediatrics, Nalanda Medical College, Patna, Bihar, India. The present study was conducted from May 2012 to Nov 2012. The study included 50 neonates being admitted to the neonatal nursery for evaluation, observation, investigation and management. The detailed general and systemic examinations of the babies were carried out. As per the proforma made, complete medical, family, antenatal and personal history was taken. Thorough physical examinations of newborn babies were done. The data generated from the present study concluded that the major congenital malformations observed were from Musculoskeletal, Central nervous System and Cardiovascular systems. Incidence of congenital malformed babies appears more now days as compared to past because of advanced diagnostic facilities and availability of neonatal intensive care unit which leads to increase chances of survival of malformed babies. The life-threatening congenital malformations must be identified by thorough clinical examination because early diagnosis and surgical correction or palliation of these infants offer the best chance for survival.