This study was conducted in order to identify the literature on oral health status and health-related QOL, review the findings systematically, and assess the association between them. We performed a literature search of reports published between January 1973 and June 2004, using five databases including MEDLINE. Only studies that used validated generic health-related QOL instruments were selected. The reviewers evaluated selected articles independently and resolved disagreements by consensus. A total of 1,726 articles were retrieved and seven were selected for the review; five observational studies and two intervention studies. Four studies showed significant associations between oral health status and health-related QOL. Temporomandibular disorders were highly associated with reduced health-related QOL. Poor oral status linked to both craniomandibular and cervical spinal pain was associated with increased impairment of health-related QOL. Dissatisfaction with the teeth and mouth, and a sensation of dry mouth contributed to reduce health-related QOL. Providing edentulous patients with implant-supported full dentures contributed to improve health-related QOL. Assessment of health-related QOL in relation to oral health with validated instruments remains insufficient. The present findings suggest that oral health status could affect health-related QOL in some settings; however, further evidence is needed to support this interpretation.