Verruciform xanthoma is a rare benign lesion. The majority of the cases occur on the oral mucosa. However, other sites, particularly the anogenital region, may be involved. We report the eleventh case in the literature of verruciform xanthoma of the penis. Genital verruciform xanthoma is significant because it can simulate verrucous carcinoma or invasive squamous cell carcinoma. Proper diagnosis by clinical recognition, adequate but limited biopsy and histopathological examination will avoid unnecessarily aggressive surgical procedures. The pertinent clinical and histological features of our case are described and the literature on penile verruciform xanthoma is reviewed.