The infant exhibited profound hypotonia after birth, thereby expanding the spectrum of <i>SCN4A</i>-related channelopathy.<h4>Methods</h4>The genetic analyses comprised targeted exome sequencing, employing a Celemics G-Mendeliome DES Panel, along with Sanger sequencing.<h4>Results</h4>Considering the clinical manifestations observed in the proband, <i>SCN4A</i> variants emerged as the primary contenders for autosomal recessive (AR) congenital myopathy 22a, classic (#620351). The gene discussed is SCN4A; the disease is congenital myopathy with cores.