Here, we report the identification of a frame-shift variant del A p.X924C at the TNPO3/Transportin-SR2 gene on chromosome 7q32.1 at position 128,597,310 (GRCh37/hg19) in all patients with limb girdle muscular dystrophy 1F. The gene discussed is TNPO3; the disease is limb-girdle muscular dystrophy.