Retroperitoneal fibrosis (RPF) is a condition characterized by the presence of inflammation and fibrosis in the retroperitoneal space, for which no standard diagnostic criteria exist. Historically, treatment has focused on relieving the obstruction with percutaneous or cystoscopic assisted placement of ureteral stents followed by more definitive resolution of ureteric obstruction with open or laparoscopic ureterolysis. However, over the past several years management has shifted from primarily a surgical approach to an immunosuppressive-based therapy aimed at modulation of the immune system. This review focuses on the recent advances in the classification, epidemiology, pathophysiology, pathology, imaging, and treatment of RPF.