In our study, SH3TC2 seems to be the most frequent gene involved in autosomal recessive demyelinating IPN, CMT4C or AR‐CMTde‐SH3TC2, as among 350 patients tested with IPN NGS, 13 had a pathogenic variants in this gene, and four patients were reported deaf. The gene discussed is SH3TC2; the disease is bile duct papillary neoplasm.