Extramammary Paget's Disease (EMPD) is a rare malignancy of the skin, primarily affecting the genital and perianal regions in older adults, often linked to underlying internal malignancies. Recent advancements in research emphasize improved diagnostic methods, including multiple biopsies and immunohistochemical markers like TRPS1, which aid in distinguishing EMPD from similar conditions. Evidence-based clinical guidelines recommend surgical resection, particularly Mohs micrographic surgery (MMS), as the preferred treatment due to its lower recurrence rates compared to wide local excision (WLE). Non-invasive EMPD may benefit from therapies like imiquimod, a topical immune response modifier, which has shown promise in achieving remission despite side effects such as fatigue and headaches. These developments underscore the importance of personalized treatment strategies and ongoing evaluation of patient outcomes to improve the management of EMPD.