The purpose of this study is to show the case of idiopathic retroperitoneal fibrosis, which clinically manifests itself as hypertension. The property of this disease described for the first time by Ormond is pathological deposition of connective tissue in retroperitoneal space. In this particular case hypertension develops as the result of renal hypoperfusion and subsequent activation of the renin-angiotensin-aldosterone system, caused by hydronephrosis and compression of renal vessels by pathologic tissue. The early diagnosis based on computer tomography and magnetic resonance provided the possibility of proper treatment. However, therapy procedure limited only to hypertension treatment may effect in progression of hydronephrosis and renal insufficiency.