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Idiopathic retroperitoneal fibrosis (RPF) is one of the causes of obstructive uropathy. This disease, ever since its original description in 1948, has undergone much trial and error in its management. We present a case of a rare type of idiopathic RPF causing obstructive uropathy, which is perirenal and different from the classical RPF seen around the great vessels. To our knowledge, such a type of RPF has not been described. We treated the patient by a combined surgical approach in the form of ureterolysis and omentoplasty, followed by medical management with steroids and tamoxifen. The combined regimen yielded excellent results, and the patient is doing well at 9 months of follow up. The rarity of the presentation, with predominant bilateral perirenal fibrosis, and response of perirenal RPF to steroids and tamoxifen, is highlighted in this case presentation.

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