Verruciform xanthoma is a rare, benign lesion classically presenting on the oral mucosa or genital area. The etiology is not yet completely understood; however, verruciform xanthoma is often associated with (a) conditions of chronic inflammation or trauma, such as lichen sclerosis, recessive dystrophic epidermolysis bullosa, and pemphigus vulgaris, as well as in a setting of (b) chronic lymphedema, (c) chronic graft versus host disease, or (d) congenital epidermal nevi, such as those associated with the Congenital Hemidysplasia with Ichthyosiform nevus and Limb Defects (CHILD) syndrome. We report a case of a solitary verruciform xanthoma on the forearm of an 82-year-old man without history of chronic dystrophic skin disease or syndrome. In addition, a thorough literature review of extra-oral and extra-genital verruciform xanthomas is presented. On the basis of this review, we believe this case is an extremely rare presentation of a solitary verruciform xanthoma on the upper-extremity of an otherwise healthy individual.