Background: Birth defects, encountered frequently by pediatricians, are important causes of childhood morbidity and mortality. Objectives: The work is to detect the frequency of the occurrence of congenital anomalies in neoborn admitted to NICU in Benghazi – Libya in the previous 5 years (2012-2016). Patients & Methods: Studied all neonate admitted during the study period from (2012-2016). Data were collected with review of records in all files of neonates born with congenital anomalies in Jumhoria hospital and Benghazi Medical Center along the duration between January 2012 and the end of 2016. Results: Demographic data for newborns studied showed that most cases with congenital malformations were male (66.2%) and females (33.8%) of all neonates with congenital malformations. Mothers ranged in age from 19 to 49 years. The average age of the mother was 35 years. Conclusions: Most frequent congenital malformation in newborn admitted to NICU in Benghazi – Libya in the previous 5 years (2012-2016) was congenital heart disease (CHD) followed by congenital neural tube defects. The year 2016 had the highest annual rate of congenital malformations.