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In this review article, we focused on the imaging features and diagnosis of infected aortic aneurysms and inflammatory diseases involving the aorta. The imaging features of other inflammatory diseases involving the aorta resemble those of infected aneurysms. In particular, chronic periaortitis is a manifestation of immunoglobulin G4 (IgG4)-related disease, which is a newly recognized syndrome of unknown etiology that has attracted much attention. IgG4-related disease is characterized by a fibroinflammatory condition with a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells. Considering their similar imaging features, infected aneurysms should be carefully differentiated from chronic periaortitis. The treatment options for these 2 potential diagnoses are completely different and consist of either corticosteroids or antibiotics. Medical history, severity (high or low grade) of fever, physical examination, clinical course (rapidly or slowly progressive), repeated blood cultures, multiple imaging modalities, and elevated IgG4 levels in addition to pathological findings would be useful in obtaining a differential diagnosis in patients suspected of having either of the aforementioned conditions.