The NM_001005361.3:c.1567A>G variant in DNM2 is a missense variant predicted to cause substitution of arginine by glycine at amino acid 523 (p.Arg523Gly). The variant is absent from gnomAD v4.1.0 meeting PM2_Supporting. DNM2, in which the variant was identified, is defined by the ClinGen Congenital Myopathies VCEP as a gene that has a low rate of benign missense variation and where pathogenic missense variants are a common mechanism of disease. The Z-score in gnomAD v4.1.0 is 4.87 (PP2). The REVEL computational prediction analysis tool gives a score of 0.713, which is above the threshold necessary to apply PP3. This variant has been reported in at least three probands with centronuclear myopathy (PS4; PMIDs: 22396310, 25501959; Genetic Services Laboratory, University of Chicago, ClinVar: SCV000193041.1). In summary, the variant meets the criteria to be classified as likely pathogenic for autosomal dominant centronuclear myopathy. ACMG/AMP criteria met, as specified by the ClinGen Congenital Myopathies VCEP: PS4, PM2_Supporting, PP2, PP3 (ClinGen Congenital Myopathies VCEP specifications version 1.0.0; 6/9/2025).