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Paget's disease of the breast generally affects middle-aged and older females. In the majority of cases, it is considered to be the result of epidermal spread from a contiguous mammary duct carcinoma. We report the first case in an adolescent female with an accessoiy nipple. The histomorphology and immunophenotypic findings support the hypothesis of in situ transformation and suggest a histologic spectrum exists in terms of cytologic atypia.