The first case of a myxoid variant of lipomatous pleomorphic adenoma arising in the intraoral minor salivary gland is presented. A well-encapsulated tumor was composed almost entirely of myxolipomatous tissue with honeycomb-like spindled cellular areas, which contained only a scant glandular element. Immunohistochemistry confirmed the myoepithelial nature of spindle cells intimately admixed with mucoadipose component. We propose the term myxolipomatous pleomorphic adenoma for this peculiar lesion.