Vulvar vestibulitis syndrome (VVS) is one of the most common causes of genital pain and pain with sexual intercourse, affecting up to 15% of women. The syndrome is characterized by severe pain on vestibular touch or attempted vaginal entry, exquisite tenderness to palpation with a cotton swab, and often subtle physical findings confined to vestibular erythema. The etiology of VVS is unknown. However, it is probably best to consider VVS as a chronic local inflammatory condition with a wide variety of etiologic causes. The heterogeneity of potential etiologies for VVS results in the use of multiple treatment regimens in clinical practice. Scientifically rigorous studies are sorely needed to determine the best treatment approach.