We describe a 13-year-old girl with juvenile rheumatoid arthritis who developed obstructive uropathy and renal failure. Retroperitoneal fibrosis (RPF) was confirmed by surgery. Although the renal failure and hydronephrosis resolved after surgery, the symptoms of vascular occlusion persisted. We consider that early diagnosis and treatment are essential. In cases of autoimmune disease, RPF should be considered when there is acquired obstructive uropathy accompanied by vascular occlusion syndrome.