Limb‐girdle muscular dystrophy R9 (LGMDR9, formerly known as LGMD2I), caused by variants in the fukutin‐related protein (FKRP) gene leads to progressive muscle weakness of the shoulder and pelvic limb‐girdles and loss of motor function over time. The gene discussed is FKRP; the disease is autosomal recessive limb-girdle muscular dystrophy type 2I.