Since 1872, 40 cases of ectopic mammary gland tissue in the vulva have been reported in the literature. Out of these, 12 had a primary cancer in the ectopic breast tissue. Seven metastases of an orthotopic breast cancer have been found in this location. We are presenting the 20th case of cancerous breast tissue in the vulva whom we classified as the 13th case of primary cancer based on clinical and histopathological criteria of primary and metastatic malignant disease. Because of the advanced age of the patient, wide local excision followed by adjuvant hormonal therapy was opted for. Nineteen months after surgery, there is no evidence of recurrent disease. Due to the rarity of this entity, its management presents therapeutic dilemmas, and variable treatment strategies are being found in the literature. In our opinion, the same basic principles used for treatment of cancers of the orthotopic breast should be applied in ectopic breast carcinoma.