Fibrodysplasia ossificans progressiva (FOP), characterized by congenital 
malformation of bones, is an autosomal dominant disorder. This is a rare genetic 
disorder and its worldwide prevalence is approximately 1/2,000,000. There is no 
ethnic, racial, gender, or geographic predilection to FOP. It is regarded as one 
of the intractable disorders, which is not only an extremely disabling disease 
but also a condition of considerably shortened lifespan. Although the genetic 
defects of FOP are not completely known, several clinical and animal model 
studies have implicated that mutations in bone morphogenetic proteins, their 
receptors, and activin receptor type IA (ACVR1) genes are associated with FOP 
primarily. The noggin (NOG) gene has also been reported in some studies. In most 
of the cases of FOP, the mutation was found as 'de novo' however there is 
paternal age effect on mutations. Unfortunately, at present there is no 
efficient treatment for FOP. The recent discoveries of genetic basis of FOP 
provide a clue to the underlying pathophysiology and potential therapy. This 
review article focuses on the genetic mutations in FOP, their usage as 
diagnostic markers, and possible target specific drug development to treat FOP 
patients.