Adenocarcinomas of the lower genital tract are rare diseases, and most of them arise from the Bartholin glands. Villoglandular adenocarcinoma of intestinal type is a very uncommon neoplasm of unknown origin with only few cases described on the vulva and in the vagina. It is characterized by villoglandular architecture, mucinous-type epithelium with intestinal differentiation (goblet cells), and direct apposition of the tumor with the surface epithelium. We report a case that developed on the hymen of a 64-year-old woman and discuss its possible origin as arising de novo from the squamous epithelium.