To provide a clinicopathologic review of vasculitis confined to the female genital tract, we describe three cases, and we searched PubMed from 1965 to 2006 with analysis of all relevant articles. We identified 118 additional cases in the literature of whom 108 had isolated necrotizing vasculitis similar to classical polyarteritis nodosa (PAN-type), and 10 presented isolated giant cell arteritis (GCA-type) of the female genital tract. In most cases, arteritis was discovered surprisingly. The mean age of these patients was 48.6 years for the PAN-type and 64.1 for the GCA-type. Vasculitis affected a single organ in 88 (81.5%) cases of which 71 (65.7%) involved the cervix in the PAN-type, whereas it was limited in the myometrium in five (50%) cases in the GCA-type. There was no progression to a systemic vasculitis in 99.1% of the cases. A comparable favorable outcome was reported after surgery in all cases with a mean follow-up of 40.8 months. We conclude that isolated vasculitis of the female genital tract is a distinct condition, with two different patterns but a similar favorable outcome after resection. Its knowledge is needed to avoid aggressive evaluation and therapy.