Introduction. A prenatal screening was introduced in the Russian Federation in 2000. It is considered as the main method of a secondary prevention of congenital malformations at the population level. The aim of this study was to evaluate the diagnostic efficiency of a prenatal screening in the Arkhangelsk region in 2012-2015 by using the data from a population-based birth registry. Materials and methods. A retrospective cohort registry-based study was carried out. We use data from the Arkhangelsk County Birth Registry (2012-2015) and 55,484 pregnancy outcomes were included in the study. Data from the birth registry were supplemented by data on pregnancy terminations followed by prenatally diagnosed birth defects (N = 273). We calculated sensitivity, specificity, positive and negative predictive values by comparing prenatal and postnatal diagnoses. Results. Congenital malformations were diagnosed in 905 fetuses (including terminations of pregnancy) in 2012-2015, that is 47.2% of all postnatal cases of postnatal or 16.3 per 1000 pregnancies. A sensitivity of the screening was 25.9% (95% CI: 24.0% - 27.9%), a specificity was 99.2% (95% CI: 99.2% - 99.4%). The maximum sensitivity was found for congenital abnormalities of the nervous system (62.7%) and chromosomal disorders (48.7%). A positive predictive value was 54.9% for all birth defects (95% CI: 51.8% - 57.9%), a negative predictive value - 97.4% (95%CI: 97.3% - 97.5%) respectively. Conclusion. Less than half of all birth defects were diagnosed before delivery in the Arkhangelsk region in 2012-2015, but 86.1% of incompatible with life defects were detected prenatally and these fetuses were eliminated. This had an impact on the reduction of perinatal and infant mortality rates in the region.