Extramammary Paget disease (EMPD) is a rare cutaneous malignancy most commonly affecting the genitals, perineum, and perianal area of the elderly. Despite its rarity, to those impacted, the disease and its treatment can have a tremendous impact on quality of life. Commonly confined to the epidermis, EMPD can be invasive, associated with contiguous extension or upward pagetoid spread of underlying malignancy or with distant synchronous malignancy. Because of its association with other cancers, formal evaluation is warranted. Treatment varies widely and evidence-based approaches are lacking. The authors review the workup and management options for the patient with EMPD.