Lichen sclerosus (LS) is a chronic inflammatory skin disease presenting mainly on the anogenital area. The relationship between female genital LS and squamous cell carcinoma (SCC) has been established, with a lifetime risk of 4% to 5% for SCC development on female patients.1 Vulvar malignant pleomorphic adenoma, also termed carcinoma ex pleomorphic adenoma, is a rare tumor, with only 2 cases reported previously.2,3 The benign counterpart, pleomorphic adenoma (PA), is a commonly diagnosed benign tumor in the salivary glands but may also occur at a variety of other sites. Only about 10 cases of vulvar PA have been reported in the literature.4 There are no previous reports of PA or malignant PA in a patient with LS. Here we report a third case of vulvar malignant PA, and the first, to our knowledge, in a patient with LS.