Klippel-Feil syndrome, the triad of limitation of movement of the head, short neck, and low posterior hairline, is often associated with other congenital anomalies. Deafness is the second most common associated abnormality and has been estimated to occur in up to 30% of patients with Klippel-Feil syndrome. Histological and roentgenographic studies suggest this deafness to be secondary to abnormal development of the inner ear. We report a case of Klippel-Feil syndrome and deafness and illustrate the associated anatomical defects with polytomography, a relatively new radiological technique which is becoming of increased value in the evaluation of congenital and acquired deafness. The importance of early evaluation for deafness is stressed when conditions known to be associated with deafness are diagnosed.