Dysgnathia complex is a rare disorder characterized by a hypoplastic or absent mandible (micro- or agnathia), a limited mouth opening (trismus), a small tongue (microglossia) and ear anomalies. Since 1961 only 21 cases of isolated dysgnathia complex are reported in literature. Diagnosis of this disorder is difficult due to the general unfamiliarity of the disorder as well as the less apparent disparity with other known developmental syndromes of the head and neck area. A case of a female recently diagnosed with dysgnathia complex is discussed.