Introduction/Background To report the case of a patient diagnosed withectopic pagetoide vulvar lesion in the vulva with breast cancer and to conduct a literature review of the diagnosis, treatmentand prognosis in that location.

Methodology A 60-year-old patient who presented a pagetoide vulvar lesionwith breast cancer to CPMC, Algiers, Algeria. The lesionwas assessed on MRI and then surgically excised; histopathology showed Invasive carcinoma of no special type (NST) after a mastectomy for the initial breast cancer We reviewed PubMed for our search, all dates using the terms: breast cancer recurrence, breast cancer metastasis, vulva and breast cancer, metastatic vulvar cancer and vulvar cancer, ectopic localisation

Results Including our case, a total of 21 publications were listed including 9 cases of IDC, 5 cases of ILC, 2 cases of undifferentiated carcinomas, 2 cases not clinically described, 1 case of comedocarcinoma and 1 case of cystosarcomaphyllodes .The time interval between the initial diagnosis of breast cancer and the secondary vulvar localization, ranges from 4 months to 255 months.

Conclusion Hartung, in 1872, first reported a fully formedmammary gland in the left labium majus of a 30-year-old womanEven the ectopic breast tissue occurs along the milklines, extending bilaterally from the mid-axillae through the normal breasts and then inferiorly to the medial groins. In women, the inferior extensions of the milk lines transverse the vulva bilaterallyIn this case; Is it a secondary localization or an ectopic localization of an infiltrating breast carcinoma?Due to the rarity of this diagnosis, there are no establishedguidelines for the treatment of the patient. The appropriatetreatment for a primary orthotopic breast cancer of a similarstage is recommended. Our patient was treated with local excision of the vulva and adjuvant.