Unilateral hearing loss is a significant complaint that is often encountered in otolaryngology practice and if left uninvestigated, it may have dire consequences. In this case, we encountered a rare condition of a 12-year old girl who initially presented with progressive unilateral sensorineural hearing loss, with no evidence of facial palsy. Neuroimaging demonstrated stenosis of the internal auditory canal. Isolated cases of congenital primary stenosis of the internal auditory canal (IAC) is a rare condition although other temporal bone conditions may also accompany this condition. Even though most patients exhibit sensorineural hearing loss, there are also cases wherein the hearing is normal in patients with stenotic canals, leading several studies to investigate the causal link between this anatomic abnormality and deafness. Typical radiographic findings are described in this case and the relevant embryological origins of the ear are traced in detail. The association of isolated IAC stenosis and hearing loss in this case suggests a correlation between stenosis and deafness. Most of the literature is focused on the effect of IAC stenosis on the outcome of cochlear implantation. There is currently no consensus regarding the therapeutic management for these types of cases since there are only a few reports in literature.