We report two siblings with progressive myoclonic epilepsy (PME) carrying compound heterozygous KCTD7 variants: c.334C > T (p.Arg112Cys), a paternally inherited variant previously reported in homozygous form and currently classified as likely pathogenic, and c.640C > T (p.Arg214Trp), a novel maternally inherited variant currently classified as of uncertain significance. Here, KCTD7 is linked to progressive myoclonus epilepsy.