Temporomandibular joint (TMJ) ankylosis with secondary dentofacial deformities in adult patients is a severely disfiguring condition and surgical treatment of this disease remains a great clinical challenge. Treatment goals are to restore the joint function, to improve facial appearances and to correct malocclusion, as well as to re-establishing harmony among them. Currently, various surgical techniques, such as arthroplasty with or without interpositional material, orthognathic surgery, distraction osteogenesis, autologous bone or bone replacement materials graft and plastic surgery, have been described in the literature. In most cases these techniques should be used in combination to achieve satisfactory outcomes. The biggest difficulty for most clinicians is to determine the proper sequence of these procedures because no uniform treatment protocol has been established. Based on the published literature and our own clinical experiences, we have prepared this review article to provide some guidelines for the surgical management of TMJ ankylosis with dentofacial deformities in adults, which will be modified and updated periodically to provide the best treatment options to benefit our patients.