Miyawaki et al. reported that a particular subset of Japanese intracranial major artery stenosis/occlusion (ICASO) mainly diagnosed as atherosclerosis not MMD, associated with RNF213 p.R4810K variant (odds ratio, 16.8; 95% confidence interval, 3.81–74.5; p < 0.0001), suggesting that RNF213 p.R4810K variant might cause various severities of ICASO [4, 6]. The gene discussed is RNF213; the disease is atherosclerosis.