Acvr1tnR206H/+;R26NG/+;Pdgfrα-Cre (Pdgfrα-R206H) mice were produced at Mendelian frequencies (n = 168 mice scored) and lacked overt abnormalities such as the great toe malformation that characterizes human FOP. Here, PDGFRA is linked to fibrodysplasia ossificans progressiva.