Verruciform xanthoma is a benign, soft tissue lesion occurring on the orogenital mucosa. Presentation of oral lesions can be pink, yellow, or gray raised plaque or papule with granular, papillary, or verrucous in nature. There are many theories proposed for the etiopathogenesis of verruciform xanthoma. Clinical observations may be misguiding as the lesion resembles malignant and premalignant lesions. Histopathological confirmation is essential for diagnosis and avoiding radical surgery. Once excised, the lesion does not recur. Here, we present a case report of verruciform xanthoma of the tongue in a 50-year-old female patient.