We report here a 12 year- old boy presenting with typical features of CMT1 type, hearing impairment, and inguinal hernia who at the next-generation sequence analysis displayed a concomitant presence of two variants: the c.233 C>T p.Ser 78Leu of the <i>MPZ</i> gene (NM_000530.6) characterized as pathogenetic and the c.1403 G>A p.Arg 468His of the <i>MFN2</i> gene (NM_014874.3) characterized as VUS. Here, MFN2 is linked to hearing loss disorder.