This review is based on a MEDLINE search of all papers on vulvar vestibulitis syndrome (VVS) published 1995-2000. The causation, natural history and prevalence of VVS are unknown. There is no convincing evidence that VVS is the result of an infection or of an allergy. It has been proposed that it is an atypical pain syndrome but there is currently no clear evidence that this is so. The usual diagnostic criteria used in VVS are of doubtful discriminative value. Findings from biopsies of women with VVS are inconsistent. While there is some evidence to suggest that women with VVS attending clinics differ psychologically from normal controls, it is not clear whether these differences reflect the effects of VVS, are the result of patient selection or influence the development of the disease. Several treatments, including biofeedback, psychosexual treatment and surgery have been reported to be successful in some patients but there is a lack of proper placebo-controlled trials on which to base estimates of efficacy. There is a vital need for further, high-quality, research in this area.