Mammary-like vulvar carcinoma represents an extremely rare disease. Here, the authors report a case of mammary-like ductal carcinoma of the vulva. A 71-year-old woman affected by stage T2N1M0 mammary-like vulvar carcinoma underwent neoadjuvant chemotherapy, radical vulvectomy, and adjuvant antioestrogen hormone therapy. The patient is alive, 24 months after surgery, with no clinical and radiological evidence of disease. To provide a better insight in to the clinical approach for this rare disease, a review of the available literature was performed.