| Summary: | Idiopathic retroperitoneal fibrosis (IRF) is a rare disease with a better prognosis if identified earlier and treated promptly. This is thought to be secondary to an autoimmune process resulting in fibrosis, causing obstructive uropathy. Radiological aspects play a major role in diagnosis and follow up. We present a young female patient who was diagnosed with idiopathic retroperitoneal fibrosis and treated successfully. This case highlights the fact that diagnosing IRF is a result of a cascade of reasonable investigations and early diagnosis prevents irreversible renal damage. Classic radiological features with histology provide a guide to the proper diagnosis, and treatment with corticosteroids gives a marked response.
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