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An unusual case of metastatic intraepithelial transitional cell carcinoma in situ to the vulvar epithelium resembling extramammary Paget’s disease is presented in a 75-year-old woman with a history of concurrent high-grade transitional cell carcinoma in situ of the bladder. Histologically and immunohistochemically, the tumor cells resembled the in situ bladder lesion. Epidermotropic spread of transitional cell carcinoma of the bladder is extremely rare, with lesions known to occur in urethral mucosa and around urethral meatus, representing an extramammary form of Paget’s disease. The present case represents previously unreported isolated intraepithelial metastasis of a urothelial carcinoma in situ to vulvar epithelium resembling extramammary Paget’s disease. [ABSTRACT FROM AUTHOR]