Background: Middle ear pathologies are associated with and persist in individuals living with human immunodeficiency virus (HIV). Yet, limited research exists on middle ear pathologies in children living with human immunodeficiency viruses.Objective: To systematically review evidence of middle ear pathologies in children living with HIV, how it is described, measures used to describe it and other relevant information.Methods: This study was a scoping review. The data were collected from different electronic databases including PubMed, Scopus, Science Direct, ProQuest, and Web of Science. The electronic database search was conducted for articles published between January 2010 and December 2020. Keywords used for searching relevant articles included ‘middle ear pathology’, ‘middle ear disorder’, ‘children’, ‘HIV’, ‘otitis media (OM)’, ‘hearing loss (HL)’, ‘hearing impairment’, ‘paediatric’, ‘minors’, ‘infants’ and ‘HIV/AIDS’. Only articles that were published in English and reported on the middle ear function and pathologies of children living with HIV were considered.Results: A total of 350 articles were extracted through databases, but only six studies were eligible and included for further analysis. Studies reviewed suggested that middle ear pathologies in children living with HIV exist and are common. Recurrent OM, type B tympanogram, chronic OM and HL with conductive element were common. Tympanometry with a 226 Hz probe tone and air bone gap were used commonly to establish the presence of middle ear pathology.Conclusion: The findings of this study highlighted that despite the dearth of evidence in this area, available evidence indicates that children living with HIV are at increased risk of middle ear pathology. However, studies in this review have mostly used middle ear measures with poor sensitivity and specificity. Therefore, the prevalence and nature of middle ear pathologies in studies reviewed may have been underreported. Further research using sensitive measures such as wideband acoustic immittance is required. Despite the paucity of evidence, the current findings raise important clinical implications for the assessment and management of middle ear pathologies in children living with HIV.Contribution: This study makes a significant contribution to the literature regarding middle ear pathologies and HIV, particularly in children.