Oral Verruciform Xanthoma (OVX) is an uncommon benign lesion of the oral cavity. Most authors consider it to develop as a response to chronic local irritation. It usually presents as a pink, yellow or greyish flat mass occurring on the gingiva, alveolar ridge or hard palate. This paper reports to you the management of a case of Oral Verruciform Xanthoma occurring on the latero-ventral border of the tongue in a young male patient, which is a rare location for this lesion. It goes on to discuss the etiopathogenesis and clinical features of this rare lesion and emphasizes the importance of having a broad knowledge of oral pathologic lesions especially for lesions that resemble malignant or pre-malignant pathologies in the oral cavity.