Abstract: Objective: To summary the clinical features of idiopathic retroperitoneal fibrosis(IRF).Methods: The clinical features and image manifestation of 1 case with IRF were analyzed retrospectively and the related literatures were reviewed.Results: The clinical features of a 72-year-old male with IRF were poor appetite, abdominal distension and kidney function damage.The multiple fiber cord formation were detected by Colored Doppler,CT and MRI.The patient were treated with D-J tube ureteral drainage in the bilateral upper urinary tract and oral prednisone.The renal function and symptoms of patient recovered and relieved.Conclusions: The initial symptoms and clinical features of IRF are not specific, the histological and imaging examination can contribute to its diagnosis.The effect of early application of glucocorticoid is good and the effects of immunosuppressant and tamoxifen are not confirmed.In late stage, surgery is needed at the time of organ oppression and obstruction.