Rarely seen is Retroperitoneal fibrosis (RPF), which presents with anomalous tissue that has both inflammatory and fibrous features. This oddity typically arises near the sub-renal segment of the abdominal aorta and iliac arteries causing ureter obstruction. Significantly, RPF may be associated with IgG4 disease as one its manifestations. Diagnostic imaging proves invaluable in identifying PRF cases. Glucocorticoids remain at forefront treatment either alone or combined alongside other medications while surgery comes secondary if non-surgical means prove ineffective; more recently though, minimally invasive procedures have become feasible options for therapy too.