Given the precedence in the literature of other patients with MMD and liver and kidney pathology (Dibi et al., 2017; Harel et al., 2015; Pracyk & Massey, 1989), the patient reported by Harel and colleagues (Harel et al., 2015) with MMD and liver disease with a known de novo RNF213 variant, as well as the near‐identical phenotypes of our reported patients, both with de novo RNF213 variants, we are highly suspicious of a distinct hepatorenal phenotype associated with RNF213 gene variants. Here, RNF213 is linked to liver disorder.