Primary cutaneous Paget’s disease is a rare malignant tumor that occurs in postmenopausal white women and has a poor prognosis. A 74-year-old postmenopausal woman was admitted to the hospital with pruritus vulvae. The gynecological examination showed that the medial mass of the left labia majora with 3 cm × 2 cm. The patient underwent extended excision of vulva masses. Postoperative pathological diagnosis was vulvar Paget’s disease with subcutaneous invasive carcinoma. The clinical stage was IA stage of vulvar carcinoma. Eleven months later, the patient had enlarged left inguinal lymph nodes and was pathologically diagnosed as a metastatic tumor (vulvar adenocarcinoma). Patients were followed up for 2.5 months without adjuvant therapy. Currently, surgery is the first choice for vulvar Paget’s disease. Considering the high recurrence rate, close follow-up is recommended. For patients with subcutaneous infiltration, chemotherapy or local radiotherapy should be considered.