Here, we present a novel homozygous <i>MYF5</i> frameshift variant, c.596dupA p. (Asn199Lysfs*49), causing premature protein termination and presenting with external ophthalmoplegia, ptosis, and scoliosis in three siblings from a consanguineous family of Pakistani origin. Here, MYF5 is linked to External ophthalmoplegia.