The impact of single-sided deafness (SSD) is well known, particularly on children. These include difficulties in speech comprehension with background noise, localization, and spatial appreciation. The social and emotional status of the child may be compromised. In addition, parents are keen to understand the etiology and consequences of SSD. Different etiologies are correlated with SSD, and etiology identification is a crucial step toward better management and parent counseling. SSD related to neural vascular conflict (NVC) of the cerebellopontine angle is uncommon. This is the first case, up to my knowledge so far, to be reported in Saudi Arabia. A 13-year-old Saudi girl presented with a history of difficulty in hearing in the left ear, and audiological evaluation demonstrated left-sided profound sensorineural hearing loss. Upon further investigation, magnetic resonance imaging (MRI) showed a left-sided neural vascular conflict of the anterior inferior cerebellar artery (AICA) loop and the left cochlear nerve. In this case, SSD was related to NVC since clinical history, and other investigations did not justify another cause. This report will embrace the role of audiological and radiological investigation, which in turn will help with management recommendations, and help clinicians in dealing with similar challenges.