Summary Background: Growing interest in health-related quality of life (HRQoL) in children with otitis media has brought the need to study the currently available HRQoL instruments with respect to their results and their applicability in clinical practice and research of otitis media. Objective: To review existing literature on health-related quality of life research in children with otitis media with respect to: (1) the measured impact of otitis media on HRQoL; and (2) the applicability of HRQoL instruments used in research and clinical practice based on their characteristics and contents. Methods: A search was performed in EMBASE (1988–November 2004) and on NLM Gateway (1966–November 2004) for studies assessing health-related quality of life or functional health status by means of disease-specific or generic questionnaires in children aged 0–18 years with chronic or recurrent otitis media with effusion or acute otitis media. The bibliographies of the selected articles were searched manually. Results: Only 13 of the 141 retrieved articles retrieved fulfilled the criteria for inclusion. In these studies, physical suffering (pain, high fever, etc.), difficulties with hearing or speech, behavioural problems, or emotional distress were reported to be the most important problems experienced by children with otitis media. Almost all instruments applied in these studies measure functional health status instead of health-related quality of life. Data on validity and reliability of these instruments are incomplete. Conclusions: Recurrent or chronic otitis media is reported to have a substantial and negative effect on various domains of functional health status and health-related quality of life of children. The OM-6 appears to be the best available instrument to assess functional health status in children with OM in a research setting. However, the lack of true HRQoL instruments as well as incomplete data on their reliability and validity, limit both our current knowledge of HRQoL in OM and the application of current instruments in both research and clinical practice.