Introduction: Verruciform xanthoma [VX] is an uncommon benign mucocutaneous lesion of unknown etiology. This rare, harmless lesion usually presents as sessile or pedunculated, pale yellowish-to-red, papillary, granular or verrucous mucosal growth.

Case Report: A 25-year-old non-smoking female presented with a lesion on the left latral surface of the tongue with no pain, that she first noticed 2 years prior to seeking medical care.

Conclusion: Blood studies revealed normal ranges. Further clinical assessment of the patient did not reveal any associated disease. The complete excision of the lesion was performed and no problem has occurred after surgery.