Perianal Paget's disease is a rare condition characterized by an intraepidermal growth of neoplastic cells with apocrine glandular differentiation (Paget's cells), often associated with an underlying malignancy. Fewer than 200 cases have been reported in the literature over the past 20 years. Here we discuss the clinical case of a young woman who was referred to our institution for this rare disorder and briefly review the literature.