Introduction. Congenital heart defects (CHDs) are common malformations and a major problem faced by physicians in their therapeutic management. The incidence of CHDs according to WHO is 10 per 1000 newborns on average. Around 500 children with CHDs are born annually in the Republic of Moldova. To date, CHDs have the highest incidence, characterized by increased morbidity and mortality, especially during childhood. Late diagnosis of CHDs leads to many irreversible complications, sometimes sudden death, that accounts for 3-5% of newborn deaths in the first week of life, and 33% of deaths in the neonatal period (0-28 days). Aim of the study. To evaluate the incidence of CHDs in children in the Republic of Moldova, depending on gender and age. Materials and methods. It is a retrospective, cohort study. The group consisted of 665 children with CHDs (51.1%), selected from the total number of 1300 patients admitted to the Cardiology Department of the MSPI Institute of Mother and Child between January 2019 and December 2019. Patients` observation sheets were examined, a number of relevant parameters being studied, such as patients` age and gender, background, causes of the disease, diagnosis, symptoms, laboratory and paraclinical investigation protocols, treatment. Results. Out of the studied group, 452 patients (67.96%) were male and 213 (32.03%) female. There were 325 (48.8%) patients aged between 0-3 years, 150 patients (22.5%) between 4-10 years, and between 11-18 years - 190 patients (28.5%). There were 254 children (38.1%) from rural area and 411 children (61.8%) from the urban area. 113 children (17%) were diagnosed with aortic stenosis (AoST), 47 children (7%) with pulmonary artery stenosis (PS) and 27 (4%) with aortic coarctation (AoCo). In 146 children (22%) ventricular septal defect (VSD) was confirmed, atrial septal defect (ASD) - 93 (14%) patients, and 27 children (4%) with atrioventricular canal (CAV), 40 children (6%) - tetralogy of Fallot (TF), 40 children (6%) were diagnosed with persistence of arterial canal (PAC), 13 children (2%) with a single ventricle and 119 children (18%) with other combined heart defects. Conclusions. Children with CHDs have a higher incidence compared with children diagnosed with other cardiovascular diseases. About 2/3 of children with CHDs were male, and half of them were 0-3 years old. Among CHDs, the most common abnormalities were found to be DSV and ASD, followed by PAC, TF and SP.