Verruciform xanthoma (VX) is an uncommon benign condition of unknown etiology, which frequently affects the oral mucosa in adults. Other less common locations include the anogenital region and the skin. VX typically presents as an asymptomatic plaque showing a verrucous appearance. Histological examination is essential for the diagnosis and shows verrucous hyperplasia of the epidermis and xanthoma cells limited to the dermal papillae. We present herein two cases of VX and discuss the histopathological findings and possible correlation with a postulated etio-pathogenesis.