Retroperitoneal fibrosis (RPF) is an uncommon disease of unclear aetiology. The review of the literature over the past 20 years revealed 160 published cases. Till now, no accepted diagnostic or therapeutic strategy exist. Most of patients diseases progress to end-stage renal failure without pharmacological treatment. In the paper we report a case of a 58-year old man admitted to the department of urology due to body mass loss, lower-back pain, vomiting, development of oliguria and anuria and intermittent claudication. On physical examination arterial hypertension (180/100 mm Hg), peripheral oedema, tenderness of the enlarged kidneys and lower limbs ischemia were found. Creatinine serum level (Pcr) was 232 micromol/l (2.69 mg/dl). On ultrasonography, symmetrical hydronephrosis and the existence of a hypoechogenic mass along of the aorta and below of renal arteries was found. The diagnosis of RPF was confirmed with MRI. Ureteral catheters were inserted with subsequent decompression of both kidneys and the patient was discharged from the hospital. Seven months later he still presented symptoms of lower limbs ischemia, arterial pressure was high and Per decreased to 138 micromol/l (1.55 mg/dl). The patient was admitted to the department of internal diseases. The treatment with prednisone at the dose of 40 mg/d during 6 weeks was introduced, and the dose was decreased gradually to 10 mg/d within 6 months. Simultaneously, the patient received intravenous therapy with cyclophosphamide 600 mg/infusion once monthly during 6 months. Two month after starting immunosuppressive treatment the intermittent claudication disappeared and after six months MRI examination demonstrated the regression of RPF. The ureteral catheters were removed. After 18 months of follow up, no recurrence of RPF is observed and the kidney function is normal.