The authors present a case report of a 4-year old girl with congenital multicystic dysplastic right kidney and congenital spondyloepiphyseal dysplasia who in a short period of time presented with hypertension crisis unresponsive to high doses of anti-hypertensive drugs. For rapid onset of secondary organic changes due to hypertension it was necessary to perform right kidney nephrectomy from vital indication.
Blood pressure normalized after nephrectomy.