Clinicopathologic findings in 8 patients with Paget's disease of the vulva are presented with a discussion of its pathogenesis. In 7 cases the disease was intraepithelial, and in 1 it was accompanied by underlying adenocarcinoma. The treatment of choice is surgical. The importance of early diagnosis, adequate excision, and close followup is emphasized. In 2 cases, the disease was associated with carcinoma of the breast and in 1 case with carcinoma of the liver.