Conclusion
SJS is a rare genetic syndrome with only a few reports of oral findings in the literature. The patient presented with typical features of SJS includes myotonia, short structure, abnormal gait, mask-like facie, blepharophimosis, microstomia, retrognathia and pursed lip. In addition, he was also diagnosed with a severe Obstructive Sleep Apnea which affected his daily activities and quality of life. We reported his oral findings that included severe dental crowding and narrow maxillary and mandibular arches. This is also the first study describing detailed craniofacial characteristics of a patient with SJS. The clinical findings of this patient suggest that SJS could have effects not only on general but also on craniofacial skeletons. And myotonia of craniofacial muscles could results in a diminished maxillomandibular development. Since maxillary and mandibular hypoplasia is a risk factor for pediatric OSA. patients with SJS could be at risk to develop OSA as the patient in this report.