Extra-mammary Paget’s disease, a rare neoplasm, have variable rates of invasion. Vulva is the most common extra mammary site, others being perineal, perianal and scrotal region in a few cases. Presence of Paget’s cells in tissue biopsy confirms the diagnosis, whereas invasion beyond basement to surrounding structure is associated with malignant nature of disease. It commonly occurs in postmenopausal, elderly females. This case reports told off a 71-year-old woman presented with an ulcerated lesion in vulva for 1-year duration which came out as invasive vulvar Paget’s disease. Wide local excision followed by post-operative whole pelvic irradiation was given for radical cure. Unfortunately, patient had local reoccurrence within 1-year and was given metronomic chemotherapy with oral cyclophosphamide and capecitabine primarily. As disease was progressive in this regimen, she was shifted to metronomic chemotherapy with oral methotrexate and celecoxib. Patient had got symptomatic relief with current regimen and is continuing the regimen for last 3-years. This case report showed the high chance of reoccurrence in vulvar Paget’s disease and partial response to oral methotrexate in such condition. More case reports and case series are needed for driving the definite conclusions on treatment protocol and role of oral metronomic chemotherapy for invasive vulvar Paget’s disease

Keywords : Vulvar Paget’s Disease; Wide Local Excision, Whole Pelvic Irradiation, Metronomic Chemotherapy