Congenital internal auditory canal stenosis is a rare cause of sensorineural hearing loss in children. A retrospective analysis including clinical manifestation and radiological findings was made for seven patients who were diagnosed with congenital internal auditory canal stenosis from 1996 to 2002. Chief presenting symptoms were hearing loss, facial nerve palsy, dizziness, and tinnitus. Hearing loss including deafness was found in five cases, vestibular function loss in four cases, and profound functional loss of facial nerve in two cases. In all cases, the diameter of the internal auditory canal was less than 2 mm on high-resolution temporal bone computed tomography (CT) scan. Two cases revealed bilateral internal auditory canal stenosis, and others were unilaterally involved cases. Congenital internal auditory canal stenosis can be an important cause of sensorineural hearing loss, facial nerve palsy, and vestibular dysfunction. High resolution temporal bone CT scan and magnetic resonance (MR) imaging were important tools for diagnosis.