Vulvar Paget’s disease (VPD) is the most common extramammary Paget’s disease (EMPD). VPD is a rare and underrecognised disease. The aetiology of primary VPD is unknown. The studies on tumour microenvironment in VPD suggest immunosuppression as a key factor. VPD is usually an intraepithelial lesion that arises from intraepidermal pluripotent stem cells in the infundibulo-sebaceous unit of hair follicles and adnexal structures. In the majority of cases, the lesion remains limited to the epidermis and mucosa without invasion and usually shows indolent disease progression. However, once Paget cells invade deeply into the dermis, regional lymph nodes and distant metastases frequently develop. Invasive cancer has been reported in 12% of women with VPD. Clinically, Paget’s disease appears as a slowly spreading ulcerative eczematoid lesion of the vulvar skin. Lesions are usually multifocal and can develop anywhere on the vulva, although they mainly occur in the labia majora. A differential diagnosis can be extremely difficult because the main clinical signs and symptoms of VPD are similar to those observed in women with squamous VIN, Lichen sclerosus, or other clinical conditions. Currently, there is no consensus regarding the optimal management of VPD. Surgery is the first treatment of choice in most cases. Depending on the site and extent of the lesion, the surgical options vary from wide local excision to radical surgical procedure, sometimes requiring a reconstruction flap. Several conservative treatments have been described as an alternative to surgery in selected patients. However, nonsurgical therapies, such as radiotherapy, chemotherapy, topical imiquimod, photodynamic therapy, and laser CO2 therapy, have limited success rates and can be considered in cases of unresectable, recurrent, and metastatic disease. Molecular markers implicated in cell cycle transitions seem to be related to prognosis and could help to tailor conventional treatments. By now, consistent preliminary data exist on hormonal receptor expression, ERBB2 amplification/overexpression and abnormal vascular proliferation, offering a concrete possibility for target therapy trials.