This relatively rare disease is most frequently reported in the skin of the perineum (peri-anal, vulvar, scrotal and upper thigh regions), but has been reported in the axilla, on the abdominal wall, and even on the tongue. It is diagnosed by biopsy. It may be associated with an underlying invasive malignancy, which should be excluded by investigation before treatment of the Paget's Disease is undertaken. Extra-mammary Paget's Disease has a significant propensity for recurring locally after surgery or radiation therapy, and has occasionally been reported to recur in grafted tissues used to replace surgical excision defects.

Wide local excision has been the main treatment recommendation for many years, with micrographic surgery (Mohs Technique) being reserved for larger lesions. Radiation therapy is certainly an acceptable alternative in situations where surgery might be functionally destructive, (e.g. in the perianal area), or when the disease has recurred after excision. Topical therapy is not recommended.​