<p>This study assessed the prevalence and clinical characteristics of Behçet’s disease (BD) in the Middle East and North Africa (MENA) region, providing updated pooled estimates and identifying gaps in current epidemiological knowledge. We conducted a systematic review and meta-analysis of studies reporting the prevalence, epidemiology, or clinical features of BD in the MENA region, published in PubMed, MEDLINE, and Web of Science databases from 2014 onward. The eligible studies had observational designs and diagnoses based on internationally validated criteria. The study quality was assessed using the AXIS and MINORS tools. Pooled prevalence estimates and clinical feature proportions were calculated with 95% confidence intervals (CI); heterogeneity was quantified using <i>I</i>². Ten studies (sample size, 31–5218) from seven countries were included. The reported prevalence ranged from 0.06% in Turkey to 0.66% in Jordan. The pooled prevalence was 0.003 (300/100,000 population; 95% CI 0.001–0.005; <i>p</i> = 0.004) with a high heterogeneity (<i>I</i>² = 87.3%). No significant sex difference was observed in disease risk (odds ratio 1.55, 95% CI 0.59–4.05; <i>p</i> = 0.374). The pooled prevalence of major clinical features showed oral ulcers, genital ulcers, ocular involvement, and positive pathergy tests in 98.7%, 73.7%, 36.6%, and 54.6% of patients, respectively. BD prevalence in the MENA region remains among the highest worldwide. Clinical manifestations are consistent with global patterns but show regional variability, highlighting the need for standardized reporting, consistent epidemiological methodologies, and targeted strategies to support early diagnosis and recognition of the disease.</p>