The review is devoted to the development of end-stage renal failure (ESRD) in children with congenital anomalies of the kidneys and urinary tract (CAKUT syndrome. In this regard, measures to slow the progression of chronic kidney disease (CKD) are important. One of the independent risk factors for a decrease in excretory renal function is arterial hypertension (AH), the correction of which is a mandatory component of nephroprotection. CAKUT syndrome is associated with a congenital decrease of the mass of active nephrons, which inevitably leads to hyperfiltration, the main cause of the development of ESRD has a high prevalence in CACUT syndrome and plays a special role in maintaining hyperfiltration. The presence of cysts, nephrosclerosis sites, and endothelial dysfunction contribute to the activation of renin secretion with a further increase in the role of the renin-angiotensin-aldosterone system (RAAS). Angiotensin II is not only a powerful vasoconstrictor. Its effects on sympathetic stimulation of dense spot, hyperplasia of smooth muscle cells, increased expression of several growth factors, cytokines and chemokines is known. The result of the interaction of this complex is the development of glomerular hypertrophy, tubulointerstitial inflammation and fibrosis. Blood pressure correction should begin with lifestyle modification. It is recommended to start taking antihypertensive drugs in children with blood pressure > 90 percentile. Among the first-line pharmacological drugs for the treatment of hypertension, angiotensin converting enzyme inhibitors (ACE inhibitors) and angiotensin II type I receptor blockers (ARBs) play a major role. Unlike adults, a combination of them is possible.