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Imaging may provide the first lead to the clinician regarding the diagnosis or possible differential diagnosis so that the targeted and focused biomarkers (blood, cerebrospinal fluid, or/and in some cases biopsies) may be obtained to clinch the diagnoses. The purpose of the article is to enumerate the causes, clinical features, and illustrate the imaging findings of the various non-atherosclerotic vasculopathic disorders and discuss "pearls" to their diagnosis. In this article, we have also discussed the latest advances in vascular ima