Three additional families were reported by Böhm et al. (2019) with prenatal‐onset muscle weakness with arthrogryposis and congenital bone fractures, and Giuffrida et al. (2019) also reported an exonic microdeletion (exons 6–9 of the ASCC1 gene) and a nucleotide variant (c.1027C>T/p.Arg343*) in a new case of SMA with congenital bone fractures 2 who was diagnosed in a stillbirth. The gene discussed is ASCC1; the disease is proximal spinal muscular atrophy.