PURPOSE OF REVIEW: Chronic periaortitis includes idiopathic retroperitoneal fibrosis, perianeurysmal retroperitoneal fibrosis, and inflammatory abdominal aortic aneurysms. This review analyses the different aspects of the disease and highlights evolving concepts concerning its pathogenesis, diagnosis, and management. RECENT FINDINGS: It has recently been reported that asbestos exposure is a major risk factor for idiopathic retroperitoneal fibrosis. An increasing number of studies showing an association with autoimmune diseases clearly support the hypothesis of a close link between autoimmunity and chronic periaortitis. Furthermore, various findings (eg, constitutional symptoms, the involvement of other organs, high acute-phase reactant levels) support the hypothesis that chronic periaortitis may be a manifestation of a systemic disease and challenge the well-known theory of a local immune response to antigens in atherosclerotic plaque. In addition to CT and MRI, which are the diagnostic modalities of choice, positron emission tomography may be useful in monitoring disease activity and response to therapy. Although there is a lack of prospective randomized trials, recent studies have highlighted the role of steroids, immunosuppressive agents, and tamoxifen in the medical treatment of chronic periaortitis. SUMMARY: Chronic periaortitis is a rare disease with protean manifestations but, if correctly diagnosed, can be successfully managed. It should be approached in the setting of a systemic process, and clinicians must be aware that other organs may be affected. Its clinical course is chronic-relapsing, so a careful follow-up is essential. Further studies are needed to investigate the pathogenetic mechanisms and the most appropriate therapeutic options.