We herein report a 63-year-old woman who presented with about 20 mm-sized mass in the right breast and the right nipple with erosion. Preoperative examinations revealed a diagnosis of HER2-type pagetoid carcinoma with axillary lymph node metastasis. After neoadjuvant chemotherapy(pertuzumab, trastuzumab, and docetaxel, followed by adriamycin and cyclophosphamide), a pathological complete response was achieved. The patient was treated with anti-HER2 therapy without recurrence.