Fibrodysplasia ossificans progressiva is a connective tissue disease. The prompt diagnosis is of utmost importance since surgical interventions are not indicated except in rare cases. We present a 5-year girl admitted due to the swelling on the back of her neck developing on her right scapula and shoulder during the recent two mounts. The course of disease was progressive in which gradually resulted in limitation of the range of motion of shoulder joint and neck. There were some other clinical findings such as ossification in distal port of the right femur and increased uptake in bone scanning at the same time.