Retroperitoneal fibrosis is a rare underlying condition that was first reported by Ormond in 1948. CT is the best choice for the diagnosis of hydronephrosis and retroperitoneal masses, and in atypical cases a biopsy of the retroperitoneal mass is required. There are no guidelines for retroperitoneal fibrosis. The following views are available in the literature: 1. Surgical treatment alone can only relieve hydronephrosis and cannot stop the progression of the disease or relieve the patient’s systemic symptoms. 2. Prednisone based drug therapy is preferred. 3. tamoxifen is not as effective as prednisone and is not recommended alone. 4. MMF (mycophenolate) combined with prednisone has achieved better results, but there is a lack of case-control study findings.5, D-J tube is the preferred way to release the obstruction, and can be removed after the general condition improves and the retroperitoneal mass shrinks. The pathogenesis of retroperitoneal fibrosis is unclear, the treatment options are immature, certain breakthroughs have been achieved, the disease has a long course, the healing process is still possible, early diagnosis and treatment is the key.