Congenital anomalies of the kidney and urinary tract (CAKUT) occupy a leading place in the structure of nephro-urological pathology and are often accompanied by changes in the cardiovascular system, which requires an interdisciplinary approach in the management of this category of patients. Purpose: assessment of the nature and frequency of cardiovascular disorders in children with CAKUT to optimize their follow-up monitoring. The study included 190 children with CAKUT, of which 24 (15.0 %) had duplicated ureters; 30 (18.75 %) — hypoplasia; 40 (25.0 %) — cysts; 30 (18.75 %) — an autosomal dominant type of polycystosis, 36 (22.5 %) — varying degrees of severity of hydronephrosis, and 30 practically healthy children without kidney pathology (group 2). The age of children by groups: 34 children were up to 3 years, 41 children — 3–6 years, 59 children — 7–11 years, and 26 children — 12–15 years. In group 1, there were 73 (45.6 %) boys and 87 (54.4 %) girls aged 4 to 16 years (mean age 8±5.82 years), and in group 2, there were 13 (43.3 %) boys and 17 (56.7 %) girls aged 4 to 15 years (average age 6±6.73 years). A comprehensive examination of the urinary and cardiovascular systems using ultrasound diagnostics and Holter monitoring of pulse, ECG, and blood pressure was carried out. Statistical processing was performed using Statistica 8.0 for Windows (Statsoft Inc., USA). Conclusions. With CAKUT, chronic infection was often recorded in children with duplicated ureters (91.7 %) and hydronephrosis (88.9 %); persistent and increased crystalluria was noted in every third patient (33.3 %) with duplicated ureters, in 47.2 % of children with hydronephrosis, and in almost 2/3 of patients (73.3 %) with polycystosis. In children with CAKUT syndrome, CHD was detected in 15.0 % of cases; the frequency of small developmental heart anomalies is high with the prevalence of left ventricular false tendon (38.9 %) and mitral valve prolapse (33.6 %). This justifies the need to include an examination by a pediatric cardiologist 1–2 times a year with an additional examination of the cardiovascular system (ECG, heart ultrasound, and daily monitoring of blood pressure) in the standard of follow-up monitoring of children with CAKUT.