From 2005 on, the phenotypic spectrum of POMT1-dependent diseases was expanded as milder forms of limb girdle muscular dystrophy (LGMD2K, 1; MDDGC1; OMIM 609308) and congenital muscular dystrophy (CMD type B1; MDDGB1; OMIM 613155) were described [7–10]. The gene discussed is POMT1; the disease is congenital muscular dystrophy due to LMNA mutation.