This systematic review with meta-analysis explores the global demographic and etiological variations of retinal vasculitis (RV), focusing on differences in frequency, age, sex, and etiology across diverse geographic populations. RV is an inflammatory condition that can lead to visual impairment, making understanding its variations essential for targeted screening and management. Systematic searches were conducted in multiple databases up to February, 2023, following PRISMA guidelines. We included studies with at least 10 RV cases, such that a frequency measurement can be estimated, without restrictions on publication date or language. RV was categorized as Idiopathic RV in the absence of additional ocular or systemic disease, Syndromic RV for ocular involvement without systemic disease, and Secondary RV in those asssociated with systemic disease. The risk of bias was evaluated using standardized tools. A total of 95 studies, including 23,180 patients, were analyzed. The overall RV frequency among uveitis cohorts was 17 %, with European populations showing the highest frequency at 25 %. Idiopathic RV accounted for 1 % of uveitis cohorts and 38 % of RV cohorts, with significant differences across continents. Behçet disease had the highest RV frequency at 56 %. The median age of diagnosis was 33.5 years, and RV was more frequent in males (57 %). Our findings underscore the considerable geographic and demographic variability in RV, particularly in Idiopathic RV, tuberculosis-related RV, and Behçet disease, highlighting the need for tailored, region-specific, and gender-specific approaches to RV diagnosis and treatment.