From a total of 14 patients with dHMN, we identified disease‐causing variants in 5 of them: three siblings carried the c.320C>T (p.Ser107Leu) mutation in BICD2 and one individual harbored a de novo mutation in DYNC1H1 (c.917A>G; p.His306Arg). The gene discussed is DYNC1H1; the disease is distal hereditary motor neuropathy.