The first and second branchial arch syndrome (HFM) is one of the most common congenital anomaly treated in craniofacial center worldwide, second to the cleft lip and palate. The pathogenesis of the HFM remains unclear. Popular hypothesis for the pathogenesis of the HFM include gene defection, mesoderm defection and stapes artery disruption. The syndrome encapsulates congenital anomalies of mandible, orbit, the external and middle ear, facial and trigeminal nerves, surrounding soft tissues on the affected side, and extracranial manifestations. Clinical manifestations of patients vary widely. Diagnostic criteria for HFM does not exist current, and the HFM diagnosis is based on physical exam findings of hypoplasia, aplasia, or malformation of the congenital or extracranial manifestations. The most widely used classification system is the OMENS classification scheme, and which later has been modified as the OMENS+ includes extracranial manifestations. A comprehensive sequential therapy with multidisciplinary coordination is needed to optimize the treatment for HFM. We reviewed the etiology, clinical characteristics, classification, diagnosis, surgical treatments for HFM.