A case of fibrodysplasia ossificans progressiva (FOP) is presented. This uncommon connective tissue disease tends to produce progressing ectopic osteogenesis. Because there are no reported curative procedures for TMJ ankylosis occurring in this condition, a palliative surgical approach is described. Etiology, diagnosis, and prognosis of the disease is reviewed. Recent research in BMP cytokine-induced bone repair may allow new approaches to treating this debilitating disease in the future.