Colonic type villoglandular adenocarcinoma of the lower genital tract is an extremely rare condition. Its origin is not clearly understood; however, the cloacal remnants are the more accepted source for this carcinoma.We report the case of a 67-year-old female patient who presented with a 1.2 cm polypoidal nodule at the right side of the fourchette. Morphologic studies revealed a colonic type mucinous adenocarcinoma that arose from within a villous adenoma. Immunohistochemical staining showed positive cytokeratin 7, cytokeratin 20, carcinoembryonic antigen, P53, and progesterone receptor, but negative for estrogen receptor and caudal type homeobox transcription factor 2. Extensive work-up failed to reveal other primary cancers in this patient. Ultimately, she underwent a radical vulvectomy. No recurrence was seen in eight months follow up of this patient after surgery. Careful, thorough histological evaluation and clinical clues enable correct diagnosis of the rare colonic type vulvar villoglandular adenocarcinoma. Due to rarity of this tumor, its management is questionable. Therefore, additional investigation is necessary for its management.