The Klippel-Feil syndrome is a congenital anomaly characterized by the fusion of the cervical vertebrae with malformation of the atlas and axis. Radiologic studies have reported a fusion or confluency of some or all the cervical vertebrae into one homogeneuos bony mass, named block vertebra. Deafness is the second most common finding and has been esti-mated up to 30% of patients with Klippel-Fail syndrome. Recently we experienced a case of Klippel-Feil syndrome having a imperforate anus, congenital heart disease, torticollis, short nack, and anomalies of vertbrae in a 2-years old boy with congenital deafness. We present the patient's hearing with the auditory brainstem reponse(ABR), and the anomalies of cervical vertrbrae with 30dimensional CT.