Abstract: Fibrodysplasia ossificans progressiva(FOP) is a rare congenital disease characterized by progressive heterotopic ossification, causing severe immobility with multi-system involvement. The relatively low incidence rate and incomplete knowledge among clinicians of the disease often result in misuse of invasive procedures or surgical treatment, leading to the progression of heterotopic ossification. To promote the knowledge of FOP, this article presents a comprehensive review involving epidemiology, pathological mechanisms, clinical features, diagnosis, and management of the FOP.