Congenital stenosis of the internal auditory canal is a rare clinical entity. The majority of patients exhibit sensorineural hearing loss, and preserve facial-nerve function. We present a patient who manifested congenital unilateral facial palsy and ipsilateral sensorineural hearing loss clinically. Neuroimaging demonstrated stenosis of the unilateral internal auditory canal and hypoplasia of the ipsilateral trigeminal, facial, and vestibulocochlear nerves, despite normal function of the trigeminal nerves. The functional prognosis of facial and vestibulocochlear nerves was poor, and cognitive development demonstrated borderline delay.