Verruciform xanthoma (VX) is an uncommon mucocutaneous lesion of uncertain etiology. Originally thought to be limited to the oral mucosa, its occurrence in other mucosal and nonmucosal sites also has been documented. Histologically, VX is characterized by subepithelial foamy histiocytes associated with papillomatosis, parakeratosis, and dyskeratosis. Subepithelial foamy cells are lipid-containing, non-Langerhans cell histiocytes. A variety of etiologies have been proposed without much consensus, including infectious (bacterial, viral, and fungal), degenerative, reactive/ reparative, inflammatory, metabolic, reactive/ multifactorial, and immunosuppressive factors. Verruciform xanthoma of the external ear is exceedingly rare. Herein, we report a rare case of VX occurring on the earlobe at a piercing site in an immunosuppressed patient and provide a discussion of the possible pathogenetic mechanism(s).