Mucoepidermoid carcinomas are malignant neoplasms that rarely involve the skin. Composed of both mucus-secreting cells and epidermoid-type cells in various proportions, mucoepidermoid carcinomas occur most commonly in salivary glands. In this case report, we describe a high-grade mucoepidermoid carcinoma with cutaneous involvement. Although this patient was referred for Mohs’ micrographic surgery, further evaluation showed either direct or metastatic extension from the parotid gland to skin and distant metastases to the lung and bone. Despite extensive bone involvement, serum levels of ionized calcium, phosphorus, and lactate dehydrogenase remained normal. In view of the widespread metastases, the treatment plan was altered to radiotherapy and chemotherapy instead of surgery. Instructive lessons from this case include the recognition by dermatologists of this rare entity, the importance of a detailed history and complete evaluation of the patient before determining appropriate therapy, and the necessity of individualizing diagnostic tests to a particular patient. (J Am Acad Dermatol 1997;37:340-2.)