Many studies suggested that the RNF213 rs112735431 polymorphism plays an important role in the pathogenesis of moyamoya disease. We performed a statistical meta-analysis based on the collected information of 18 studies comprising of 3044 cases and 6317 controls. We also assessed the correlation between the rs112735431 polymorphism and clinical features. Pooled odds ratios (ORs) and 95% confidence intervals (95%CIs) were used to estimate the strength of associations. Fixed-effects and random-effects models were used. Result of this meta-analysis found that the rs112735431 polymorphism is significantly associated with predisposition to moyamoya disease in Asians in all genetic models (homozygote model: OR = 35.19, 95%CI = 16.07–77.08; heterozygote model: OR = 88.72, 95%CI = 52.55-149.78; dominant model: OR = 89.77, 95%CI = 54.00-149.24; recessive model: OR = 16.52, 95%CI = 7.63–35.77). The moyamoya disease patients carrying GA + AA genotype of RNF213 gene preferred to ischemia than that of wild-type subjects (OR = 2.15, 95%CI = 1.03–4.48). Our results suggested that the rs112735431 polymorphism is associated with moyamoya disease risk and may be an efficient biomarker to classify ischemia/hemorrhage phenotypes of the moyamoya disease. Well-designed large-scale multicenter epidemiological studies will be required to validate our findings.