Fibrodysplasia ossificans progressiva (FOP) or myositis ossificans is a very rare autosomic dominant disease. Progressive ectopic bone formation occurs in ligaments, muscles and fascia and provoque severe movement limitation with time. The aim of this article is a systematic review of the litterature on FOP and on related problems occurring in oral and maxillofacial region. Some guidance on FOP treatment planning in oral and maxillofacial area are also proposed.