Chronic periaortitis includes a spectrum of rare conditions characterized by fibro-inflammatory tissue surrounding the abdominal aorta. Although it has been considered a localized inflammatory disease secondary to atherosclerosis, several clinico-laboratory findings suggest a systemic autoimmune origin; additionally, it may involve the thoracic aorta and the origin of its major branches, with a pattern similar to that of the large-vessel vasculitides. Its pathogenesis is still unclear. Computed tomography and magnetic resonance imaging are the modalities of choice for the diagnosis, whereas fluorodeoxyglucose/positron emission tomography emerges as a sensitive imaging modality to assess the inflammatory activity of the periaortic tissue. The treatment of chronic periaortitis is largely empirical, since no randomized trials have been carried out. Corticosteroids, immunosuppressants and endoscopic or surgical procedures must be appropriately combined for the correct management of chronic periaortitis patients.