Verruciform xanthoma (VX), a rare, benign lesion of the skin and mucosa, is slow-growing, asymptomatic and characterized by a granular (verruciform) surface. It is yellowish-red or grey in color and up to 2 cm in diameter. Histologically, a papillary and/or verrucous proliferation of the squamous epithelium with hyperparakeratosis and numerous foam cells is present. These cells are predominantly located within the papillae of the lamina propria. For differential diagnosis, other papillomatous and verrucous lesions such as verrucous carcinomas or squamous cell carcinomas need to be ruled out.A 46-year-old patient with VX located on the alveolar process regio 26-28 is presented. Clinically, a 2 x 2 cm granular, oral mucosa surface lesion extending onto the palate occurred in regio 26-28. Biopsy was characterized light microscopically by the presence of swollen, elongated cells in the submucosa, an indication of VX alterations. Transmission electron microscopy demonstrated foam cells in the subepithelium containing numerous membrane-bound vesicles similar in diameter and showing a wide variation in electron density. Morphologically, these cells resembled macrophage-related cells. The lesion was excised in total with no evidence of recurrence after 9 months.The pathogenesis of VX is still unclear. The characteristic xanthoma cells may play a major role in VX. Microscopic analysis of the morphology of the foam cells indicated that they may represent a differentiated form of macrophages. Lipid vesicles inside these cells differed in their electron density indicating a heterogeneous biochemistry or different states of maturation.