Approximately 97% of FOP cases are caused by a single nucleotide substitution mutation (c.617G > A, R206H) in the gene coding for the Activin receptor type 1 (ACVR1)/Activin receptor-like kinase 2 (ALK2), a bone morphogenetic protein (BMP) receptor [3,4]. This evidence concerns the gene ACVR1 and fibrodysplasia ossificans progressiva.