Oral-Mandibular-Auricular Syndrome - the rare inbom deformity (its proportion ranges from 1:3500 to 1 :4500 newborn babies). This defect is characterized by the malformation of the ear, mouth and mandible in the effected side, as a result, it affects both functional and apparent patient's face. Up to now, the time and the way of treatment have been controversial. However, the treatment of mandible distraction shows many advantages. In Viet Nam, the authors reviewed medical literature and found that none of these case were reported. The authors have recently had one case in which patient was treated by preoperative mandible distraction, othronatic, othrodontic and lipofilling. The objective of this writing is to report a rare case and reviewing the literature, aiming to manage time and therapy for this abnormality.