Objective: To determine the incidence and types of congenital anomalies in a Turkish population. Method: The total number of neonates (9160) born in the Department of Obstetrics and Gynecology, Gazi University Faculty of Medicine during 1988-1995 were studied retrospectively. Newborns with congenital anomaly were identified from their birth registries. The total incidence, types and combined anomalies were determined. Also, the relationship between congenital anomalies and maternal age andior gender were investigated. For statistical evaluation, Chi-square test, Yates correction and Fisher's exact tests were used where appropriate. Results: The overall congenital anomaly incidence was 1.11% and the NTD incidence was 0.27% in our population. Anencephaly was the second most common NTDs with the ratio of 40%, following the spina bifida cases. There was a significant difference between female and male newborns with ancephalocele (P < 0.05). Urogenital system anomalies were found to be the second most common type of malformation with an incidence of 0.21%. Facial and musculoskeletal system abnormalities were the third and fourth most common malformations. Omphalocele incidence in our population was 5 in 9160 births and gastrochisis was 1 in 9160 births. Conclusion: The overall congenital anomaly incidence in newborns in our population is 1.11%. The most common malformations were CNS and urogenital abnormalities. NTDs incidence was 0.27% in a Turkish population.