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Objective. The aims of this study were to investigate retroperitoneal fibrosis in a Spanish hospital and present a review of the international literature to attempt to elucidate a diagnostic and therapeutic approach to this unusual pathology. Material and methods. A database search was performed in the pathology department and in the documentation service using the key words 'retroperitoneal fibrosis' and 'Ormond's disease', limiting the search to the years 1990-2010. Cases in which secondary retroperitoneal fibrosis was considered were excluded. In addition, a PubMed literature search was performed using the terms 'retroperitoneal fibrosis' and 'Ormond's', limiting the search to 1985-2011. Results. Twenty-two patients were diagnosed with idiopathic retroperitoneal fibrosis (IRF) or Ormond's disease. The most common symptom at the time of diagnosis was flank pain. With regard to laboratory findings, five patients (22.7%) had anaemia and eight (36.3%) had renal failure. Computed tomography (CT) was performed in 20 patients (90.9%) and the most common finding observed was retroperitoneal mass. Eighteen patients were started on corticosteroids, in six cases in association with azathioprine. Three patients had recurrence at 12, 24 and 72 months, respectively, and 15 patients required emergency surgery. Nine open surgical procedures were performed. Conclusions. At present, IRF is considered an autoimmune disease that presents with local and systemic signs and symptoms. CT and magnetic resonance imaging are the two tests of choice in IRF diagnosis and follow-up. [18F]Fluorodeoxyglucose positron emission tomography is starting to be used for assessment and treatment response. A combination of medical and surgical treatment is usually applied. It is essential to administer corticosteroids alone or in association with other immunosuppressive drugs such as azathioprine. Laparoscopic ureterolysis, or robotic ureterolysis, if available, is the technique of choice. [ABSTRACT FROM AUTHOR]