Congenital malformations (CM) in fetuses and neonates belong to main causes of perinatal morbidity and mortality. Provision organs malformation and malfunction in such cohorts is less studied. The purpose - to analyze the course of pregnancy, the data of ultrasound imaging of provisional organs in the presence of CM of central nervous system (CNS) and/or of nephro-urinary system (NUS) in the fetus and newborn. Materials and methods. The results of prenatal and postnatal ultrasound, anamnestic and general clinical data of a sample of cases with prenatally diagnosed CM of CNS and/or NUS for the period 2017-2021 were analyzed. Results. There were 45 newborns with CNS malformations, and 54 newborns with CM of NUS. Malformations of NUS and/or CNS in the examined newborns were combined with anomalies of other systems in a third of cases - 29.6% and 31.1%, respectively. According to the results of prenatal ultrasound examinations, polyhydramnios (16.7%) was most often recorded in the pregnancies with fetal CM of NUS, and cases of fetal CM of CNS most commonly were registered placental hyperplasia (35.6%), fetal growth retardation (24.4%) and fetal distress (26.7%). Postnatally in both cohorts (with NUS malformations and with CNS malformations) a high rate of following complications were recorded: prematurity (16.7% and 15.6%, respectively), birth asphyxia (48% and 55.6%, respectively), and early neonatal death (11% and 6.6%). Сonclusions. Pregnant women with CM of CNS and/or NUS in the fetus belong to the group of high perinatal risk because of the high rate of perinatal complications. Information about the identified high perinatal risks in fetuses and newborns with CM of the CNS and/or NUS should be provided to parents and taken into account when planning management of pregnancy and labor.