The purpose of this article is to outline the current state of evaluating children with unilateral hearing loss, with significant focus on cochlear implantation, in terms of reviewing the key points of the history, including the duration of deafness, outlining the recommended audiometric testing battery, and discussing issues related to imaging of the auditory system and related anatomy. In addition, a comprehensive and up-to-date summary of outcomes in terms of speech perception, sound localization, and quality of life for both the child with unilateral hearing loss as well as their parent(s) is reported.