Enlargement of the cochlear aqueduct is a controversial topic, with experienced investigators doubting its existence because of a lack of published cases. We describe the CT appearance of an enlarged cochlear aqueduct in a patient with advanced congenital inner ear anomalies and congenital hearing loss. The intent of this article is to present the CT appearance of a presumably enlarged cochlear aqueduct and to underscore the need to examine this structure in patients with congenital hearing loss.