LMNA gene mutations have been associated with several diseases. Some of them are muscle disorders, including the autosomal dominant and recessive forms of Emery-Dreifuss muscular dystrophy (EDMD2 and EDMD3), limb-girdle muscular dystrophy 1B (LGMD1B) and congenital muscular dystrophy (MDCL). With few exceptions, most of the studies are focused on one of these phenotypes and data on large cohorts of myopathic patients are lacking. The aim of our study was to evaluate clinical, neurophysiologic, histological and molecular features of 77 myopathic patients mutated in LMNA gene and followed in different Italian neuromuscular centres. Patients with predominant or exclusively progeroid/lipodystrophic or cardiac phenotype were excluded. The cohort included 36 (46.8%) patients with LGMD1B phenotype, 30 (39%) with EDMD2 and 11 with MDCL (14.3%). Mean age at onset was 10.3 ± 12.3 years in patients with EDMD2, 29.5 ± 17.6 in LGMD1B and 0.4 ± 0.5 in MDCL. Independent walking was reached in all patients, with the exception of 4 (5.2%) MDCL, who never acquired the ability to stand up. Another 4 (5.2%) patients (3 LGMD1B and 1 EDMD2) lost ambulation, and 3 EDMD2 (3.9%), required monolateral support to walk. Cardiac involvement was found in 57 (74%) patients. Pacemaker or ICD were implanted in 42 (54.5%) patients (21 EDMD2, 18 LGMD1B and 3 MDCL), while heart transplant was performed in 5 (6.5%) patients (3 EDMD2 and 2 with LGMD1B). Six (7.8%) patients died due to cardiac problems (3 EDMD2 and 3 MDCL). Only 5 (6.5%) patients required non-invasive ventilation (4 EDMD2 and 1 CMD), confirming that respiratory problems are not a major issue in LMNA-associated myopathies. Ten novel mutations were detected. Despite EDMD2, LGMD1B and MDCL phenotypes are part of a continuum spectrum, their identification is clinically and prognostically relevant.