Arthrogryposis multiplex congenita (AMC) is a rare entity gathering many diseases, isolated or included in more global syndromes. It affects symmetrically joints of lower limbs, back and maxillofacial area with temporomandibular joint (TMJ) impairment leading to deterioration of oral functions and mouth opening resulting in poor quality of life. Therapeutic strategy varies according to type and severity of impairment and could involve physical therapy and/or surgery. This article reported a case of AMC followed in our unit and highlighting various maxillo-facial impairments, especially restricted mouth opening and its consequences due to fibrotic TMJ and hypertrophic coronoid processes. We described our therapeutic approach based on surgery and postoperative physical therapy. A stable improvement of mouth opening was obtained, and patient’s quality of life was increased. We performed a review of the literature and suggested a therapeutic management of mouth opening limitation in AMC.