Klippel-Feil syndrome(KFS) is defined by congenital cervical vertebral spine fusion and is seen with a wide spectrum of dental manifestations and craniofacial profiles. Previous studies on lateral cephalograms have documented an association between fusion of the cervicalvertebrae and deviations in the craniofacial profile in non-syndromic patients with severe malocclusion. To our knowledge, no previous studies have described the craniofacial profile including the cranial base of KFS patients on lateral cephalograms. Therefore KFS and its craniofacial and dental manifestations were described according to existing literature and additionally the craniofacial profile and cranial base was analysed on lateral cephalograms of two patients with KFS. According to the literature the dental manifestations of KFS-patients included oligodontia, overjet, cross bite, open bite and deep bite. The craniofacial profile was clinically described as reduced lower facial height, midfacial hypoplasia, and mandibular prognathia. The analyses of the two lateral cephalograms showed increased mandibular inclination, increased vertical jaw-relationship, increased jaw angle and maxillary retrognathia. The cranial base was normal in both cases. The sagittal jaw relationship and mandibular prognathia varied between the two cases. The literature review and the analyses of the two lateral cephalograms have shown that deviations in the occipital and cervical spine field as KFS were associated with deviations in the teeth and craniofacial profile. Previous studies on lateral manifestations of craniofacial profiles of cervical vertebral bodies non-syndromic patients with severe malocclusion. To our knowledge, no previous studies have described the craniofacial profile including the cranial base of KFS patients on lateral cephalograms. KFS and its craniofacial and dental manifestations were described according to existing literature and additionally the craniofacial profile According to the literature the dental manifestations of KFS-patients included oligodontia, overjet, cross bite, open bite and deep bite. The craniofacial profile was clinically described as reduced lower facial height , midfacial hypoplasia, and m The analysis of the two lateral cephalograms saw increased mandibular inclination, increased vertical jaw-relationship, increased jaw angle and maxillary retrognathia. The sagittal jaw relationship and mandibular prognathia varied between the two cases. The literature review and the analyzes of the two lateral cephalograms have shown that deviations in the occipital and cervical spine fields as KFS were associated with deviations in the teeth and craniofacial profile.