OBJECTIVE: The aim of this systematic literature review was to evaluate associations between different malocclusions, orthodontic treatment, and signs and symptoms of temporomandibular disorders (TMD). MATERIALS AND METHODS: This review was part of a project at the Swedish Council on Technology Assessment in Health Care focusing on malocclusion and orthodontic treatment from a health perspective. As a first step, the literature was searched in the Medline and Cochrane Library databases from 1966 to May 2003. A later update was made in January 2005. Human studies in English or in Scandinavian languages were included. RESULTS: Associations between certain malocclusions and TMD were found in some studies, whereas the majority of the reviewed articles failed to identify significant and clinically important associations. TMD could not be correlated to any specific type of malocclusion, and there was no support for the belief that orthodontic treatment may cause TMD. Obvious individual variations in signs and symptoms of TMD over time according to some longitudinal studies further emphasized the difficulty in establishing malocclusion as a significant risk factor for TMD. A considerable reduction in signs and symptoms of TMD between the teenage period and young adulthood has been shown in some recent longitudinal studies. CONCLUSIONS: Associations between specific types of malocclusions and development of significant signs and symptoms of TMD could not be verified. There is still a need for longitudinal studies.