A histopathological study of the temporal bones of a 3-year-old black girl who had bilateral deafness associated with Waardenburg's syndrome type II showed a similar pattern of pathology in both ears. The most striking findings were an absence of pigmentation in the inner ear and cochleosaccular abnormality. This is, to our knowledge, only the third report on human temporal bone histopathology in Waardenburg's syndrome and the first report of such a case with absence of pigment (melanin) in the inner ear. A possible association of hearing loss with absence of inner ear pigment in this case is discussed.