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Although most patients exhibit a favorable prognosis, those with metastatic extramammary Paget's disease typically face poor outcomes, necessitating effective therapeutic strategies to improve survival and disease management. Due to the rarity of this condition, no standardized treatment consensus exists for the disease. In this review, we summarize current treatment modalities for metastatic extramammary Paget's disease, including chemotherapy, hormonal therapy, targeted therapy, immune checkpoint inhibition, and several emerging novel therapies, which demonstrate variable disease control rates and survival outcomes. Additionally, we explore the potential of promising therapeutic approaches for future applications. 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