RNF213 p.Arg4810Lys was significantly associated with ICAS-only, ECAS-only, and concurrent ICAS and ECAS (aOR, 3.72; 95% CI, 1.30–10.60; P= 0.014, aOR, 7.04; 95% CI, 1.51–32.77; P= 0.013, aOR, 11.68; 95% CI, 4.25-32.07; P < 0.0001, respectively; Table 3). This evidence concerns the gene RNF213 and familial isolated congenital asplenia.