Verruciform xanthoma is a benign mucocutaneous lesion with equal gender predilection and a wide age range. The masticatory mucosa is the most affected location in the oral cavity, especially the gingiva and palate. The lesions are normal-colored, although red, white, or yellow presentation has been described. They usually manifest as asymptomatic, slow-growing, well-demarcated plaques or nodules, often described as bumpy, rough, verrucoid and/or papillary lesions. Intraosseous xanthoma is a rare benign entity characterized by bone destruction of variable severity that may also cause bone expansion or cortical perforation. The biological nature of this lesion is debatable, and some authors advocate that intraosseous xanthoma may better represent a reactive or degenerative process rather than a true neoplastic entity. In the gnathic bones there is a predominance of cases among young adults, with no sex predilection, and more often affecting the posterior region of the mandible. The presence of foamy histiocytes is the main histological finding of xanthomas, and these cells are positive for mature histiocytic markers like CD68 and CD163 proteins. Verruciform xanthoma is treated with simple surgical removal, while intraosseous xanthoma is more often treated conservatively with curettage. Recurrences are very rare.