Background. Currently, the study of congenital anomalies (CA) including of the central nervous system (CNS) is of major interest for child health care. CA occur in 2 – 3% of newborns (NN) and their contribution to infant mortality is 20%. In the Republic of Moldova (RM) CA ranks second among the causes of infant mortality. Among the prophylactic measures an important place is given to CA monitoring. At international level, the European szstem of CA monitoring, EUROCAT, comprises 35 regional registries from 21 countries in Europe, according to the requirements of the register 55 isolated malformations, chromosomal abnormalities and syndromes. Aim of the study. The main aims which are attained by carrying out CA monitoring in our country, are determining the frequency of CA in the population of the Republic of Moldova and studying their dynamics and etiology. Materials and methods. Since 2009 the National Register of CA of the Republic of Moldova has been included in EUROCAT. The CA frequency is calculated according to EUROCAT formulas, as the ratio between the total number of CA cases determined to the number of newborns (NN), stillbirths and deceased children, as well as cases of abortions in the cases of CA according to medical indications, compared to the total number of live and stillbirths. Statistical method used in accordance with EUROCAT requirements. Results. The average prevalence of CA for 2012 – 2021 was 15.92 per 1000 live NN. This value in the European EUROCAT Registry is 20.2 – 20.4 per 1000 live NN. According to the data of the National Registry of CA at NN of the Republic of Moldova, during 2012 – 2021 the incidence of congenital anomalies of the CNS was 1.73 per 1000 NN. Thus, the incidence of spina bifida was 0.33 per 1,000 NN. According to EUROCAT Registry data, the incidence of congenital CNS anomalies during the investigated period was 1.22 per 1000 NN. Spina bifida was registered at 0.17 per 1000 live NN. Conclusions. The data presented including the CA frequency below 20 per 1000 NN suggest the need to improve CA registration according to EUROCAT recommendations.