Conclusion: STIC and p53-signature as precursor lesions of pelvic serous cancer is seen in macroscopically inconspicuous Fallopian tubes in unilateral TC in patients with elective BSO and patients affected by PPC. We propose that the sectioning and extensively examining the fimbria protocol be applied to all cases with PPC, TC and in women with prophylactic BSO. Objective: Primary adenocarcinoma of the vulva is rare, and enteric differentiation is excepcional. Only a few cases of neoplasms of pure intestinal-type in the low genital tract have been reported. They are considered to arise from cloacal remants or from intestinal heterotopia. Method: We present the case of a 54 year-old woman, with a histerectomy performed 10 years before. On clinical examination a polipoid lesion in the vulvar vestibule was noticed. Biopsy revealed a tubular adenoma of intestinal type with a focus of adenocarcinoma. The first histologic interpretation was a metastatic intestinal tumor. Clinical examination, recto-colonoscopy and magnetic resonance imaging of the abdomen excluded this possibility. Results: The immunohistochemical study showed reactivity with citokeratin 20 but didn't with citokeratin 7. All this led to conclude that it was a primary intestinaltype adenocarcinoma of the vulva arisen from an adenoma. Conclusion: On our knowledge there are less than 100 reported cases of intestinal adenoma in the genital tract. They have been described also in the vagina and cervix but only a few developed an adenocarcinoma. It is important to be aware of this tumor type and to distinguish it from metastatic colorectal adenocarcinoma in order to plan appropriate treatment.