The current and most prevalent molecular model proposed as causative of FSHD associates reduction in the number of D4Z4 repeats (FSHD1) or loss of SMCHD1 function (FSHD2) to hypomethylation of the CG‐rich D4Z4 sequence and transcription of the most distal copy of the DUX4 gene through the last D4Z4 unit and abutting telomeric sequence.7 This evidence concerns the gene DUX4 and Facioscapulohumeral dystrophy.