Here, we successfully generated induced pluripotent cells from a patient with limb-girdle muscular dystrophy and devise two strategies to rescue the disease-specific genomic mutations: (i) on the level of LGMD2A-iPSC, we have combined CRISPR/Cas9 genome targeting with a FACS and Tet transactivator based biallelic selection strategy which resulted in a new functional chimeric exons 3-4 without the two CAPN3 mutations. The gene discussed is CAPN3; the disease is limb-girdle muscular dystrophy.