Literature described wide disparities in incidence and prevalence between different types of vasculitis. There were no comprehensive studies on ethnic or racial associations in all types of primary systemic vasculitis (PSV) in any published article until this review commenced in 2020. The review aims to synthesize the evidence regarding the relation between ethnicity and the incidence and/or prevalence of different types of PSV. A total of 52 selected articles which include clinical trials, cohorts, cross-sectional studies, case series, and case studies and have been published within the last 10 years in the human population, were reviewed by searching Medline, PubMed, and Google Scholars databases using predefined keywords. The PRISMA diagrams were followed to identify relevant articles. The methodological qualities of the studies were assessed using the EPHPP tool. Finally, a summary of the evidence on the association between ethnic origin and PSV was painstakingly compiled. The connection between ethnicity and different types of PSV has been found to be significantly diverse in this researchas vasculitis is more common in Asians and Scandinavians, Kawasaki disease and periarteritis nodules are more prevalent in Japanese and Alaskanatives,ANCAassociated vasculitis is more frequent in Caucasians, whereas Henoch-Schonleinpurpura and Cogan syndrome more usual in Caucasians and Asians. Furthermore, Behçet's disease more commonly occurs on the "Silk Road," especially in Turkey. Genetic susceptibility and environmental elements could contribute to the global variation in the incidence and prevalence of primary systemic vasculitis.