ObjectiveThis study aimed to demonstrate the temporal bone histopathology of Cornelia de Lange syndrome.Study DesignHistopathologic examination of an infant with this syndrome.SettingThis study was performed at the temporal bone laboratory, Fukushima Medical University, Fukushima.ResultsThe authors describe interesting temporal bone findings of this syndrome: anomalies of the middle and inner ear and the facial nerves.ConclusionsThis is the second case report describing the histologic findings of the temporal bone in Cornelia de Lange syndrome in the English literature.