A case of Waardenburg syndrome type II is reported. The computed tomography (CT) revealed aplasia of the posterior semicircular canal and poor development of the vestible. Until now, including our case, only a few cases of Waardenburg syndrome with deafness were studied roentgenologically. Among them, at least 26% revealed aplasia of the posterior semicircular canal, which infers that it is a characteristic finding of Waardenburg syndrome.