Surgical treatment of temporomandibular joint (TMJ) ankylosis and its secondary dentofacial deformities seriously affects facial appearance and remains a great clinical challenge. The goals of its treatment are to restore joint function, improve facial appearance and correct malocclusion, and re-establish the relationship between the three. Various surgical techniques, such as arthroplasty with or without built-in materials, orthognathic surgery, distraction osteogenesis, autologous bone or bone replacement material grafting and cosmetic surgery, have been described in the literature. In most cases a combination of these procedures is required to achieve a relatively satisfactory result. I. Introduction to temporomandibular joint ankylosis Temporomandibular joint (TMJ) ankylosis is a joint disease in which bony or fibrous adhesions of the temporomandibular joint cause dysfunction. It occurs in childhood and can cause secondary dental and maxillofacial deformities as well as physical and psychological disorders. Temporomandibular joint ankylosis is less common in developed countries, probably due to more standardized use of antibiotics and more scientific treatment of condylar fractures. The incidence of TMJ ankylosis remains high in developing countries. To date, surgical treatment of TMJ ankylosis with odontofacial deformity remains a great clinical challenge, as this deformity is often complex and extensive. It affects not only the temporomandibular region but also the entire jaw and face. Surgical treatment may require a series of procedures. One of the difficulties for many clinicians is the inability to determine the correct sequence of these procedures. II. Etiology and clinical features of joint ankylosis The two main causes of temporomandibular joint ankylosis are trauma and inflammation. By far, traumatic TMJ ankylosis is more common. This may be due to the inability of these patients to receive effective standardized treatment, and even less access to TMJ specialist care, and the lack of appropriate treatment or no treatment at all after condylar trauma. Restricted mouth opening leads to problems with mastication, digestion, speech and oral hygiene. If the joint ankylosis occurs in childhood, it can lead to skeletal deformities involving the entire jaw and face. Whether this manifests as a facial asymmetry or a small jaw deformity depends on whether the disease is unilateral or bilateral. In unilateral cases, the jaw deformity is tilted in the plane of dentition, and in “bird’s mouth deformity”, the vertical height of the upper jaw is insufficient. The recession of the lower jaw affects the size of the oropharyngeal cavity and in severe cases even obstructs the airway. Long-term contraction of the masticatory muscles causes elongation and thickening of the coronoid process, shortening of the mandibular branch, recession of the chin, and formation of the anterior incision of the mandibular angle. Joint ankylosis and skeletal deformity can also lead to low self-esteem, aloofness, stubbornness and other psychological problems. The goal of TMJ ankylosis treatment is to restore joint function, improve facial appearance, correct malocclusion, and re-establish the relationship between TMJ, facial appearance and teeth. In particular, clinicians are required to focus on dental problems. In fact, dental substitution due to skeletal deformities is often a challenging challenge for the orthodontist as well. Restoration of good occlusion plays an important role in the stability of oral function and postoperative outcome. Therefore, the most satisfactory treatment results can only be achieved through a combination of surgical and orthodontic treatment.