Nemaline myopathy is one of the most common forms of congenital myopathy, with a prevalence that differs between studies (overall <20% of all congenital myopathies).1, 2, 3 Seven genes related to this disease encode proteins involved in muscle contraction: ACTA1, NEB, TPM3, TPM2, CFL2, TNNT1, and LMOD3. 1, 4, 5KBTBD13, KLHL40, and KLHL41 genes have also been associated with nemaline myopathy but encode proteins contributing to transcriptional regulation and degradation.1 This evidence concerns the gene KLHL40 and congenital myopathy.