Phyllodes tumors of the vulva are rare proliferations that share morphologic similarities with breast neoplasms. Their histogenetic origin is elusive and may be associated with specialized mammary-like glands of the vulva. Because of their rarity, the clinical and pathologic features, classification, and therapy are not well defined, and their biologic behavior is difficult to predict by histology alone. Immunohistochemical expression of estrogen and progesterone receptors and breast markers provide further support for a common origin. Surgical resection is the current mainstay of therapy and is definitive in most cases.