In recent years, the RNF213 gene has been identified as a disease susceptibility gene and 80% of patients with moyamoya disease have RNF213 c.14429G>A (p.Arg4810Lys, rs112735431) (based on the National Center for Biotechnology Information Reference Sequences NM_001256071 and NP_00124300), which is the most frequent gene variant observed [20,21]. Here, RNF213 is linked to Moyamoya disease.