Idiopathic retroperitoneal fibrosis (IRF) is a rare disease characterized by inflammatory infiltration around the aorta, which can lead to clinical symptoms and complications, the most common of which is ureteral obstruction, potentially resulting in secondary renal failure. A search of medical databases was conducted to compile a review article summarizing the immunosuppressive treatment options for IRF. In addition to urological treatment, immunosuppressive drugs are used in the treatment of retroperi­toneal fibrosis. The cornerstone of therapy remains glucocorticosteroids (GCs), whose effectiveness and relatively rapid effect have been proven. However, the issue of frequent disease relapses after discontinuation of GCs still remains, prompting clinicians to investigate the effectiveness of other immunosuppressive agents. The efficacy of numerous immunosuppressive and biological medications in the treatment of IRF has been demonstrated in several studies; however, studies involving larger patient groups are still needed.