A rare case of the first and second branchial arch syndrome was reported. The patient revealed a left facial hypoplasia, hypoplastic deformed small ear, and obstruction of the external acoustic meatus, and additionally, submucosal cleft palate was recognized. In the oral cavity, highly advanced many carious teeth, and cross-bite at the anterior portion were observed. Roentgenographically under development of the left mandibular ramus, deformity of the nasal septum, and enlargement of the mandibular angle were also shown.It has been generally said on the genesis of this syndrome that developmental disturbance of mesodermal components of the first and second branchial arch would occur in the approximate 7 th week of the uterine life, however in this case, the clear cause was not clarified.The patient was operated in the manner of usual cleft palate plastic surgery and has been trained in the speech therapy.