Two TPM2 mutations, i.e., c.349G>A, p.Glu117Lys and, c.364G>A; p.Glu122Lys, were identified in an unspecified congenital myopathy, diagnosed in the patients without specific structural alterations in the muscle biopsy specimen (Brandis et al. 2008; Donner et al. 2002). Here, TPM2 is linked to congenital myopathy.