This recessive myopathy should be considered a distinct TNNT3‐related clinical entity not only for the different genotypes of affected individuals but also because of the progressive course and the presence of clinical manifestations that are absent in DA2B2, such as severe weakness, hypotonia, and abnormal muscle histology [2, 5, 12, 13, 20]. Here, TNNT3 is linked to myopathy.