A non-smoking man in his seventh decade presented with a lesion he had had for about 5 years on the right external surface of the lip 1 mm from the vermilion border. On physical examination, a yellowish-coloured, non-tender, verrucous mass of 0.5 x 0.7 cm was observed. A histopathological examination of the first 3-mm punch biopsy was non-diagnostic. The histopathological evaluation of a second, 5-mm-deep punch biopsy made by a pathology expert in oral lesions revealed a diagnosis of verruciform xanthoma. The key to the diagnosis of verruciform xanthoma is to recognize xanthoma cells. However, these cells may be insufficient in number in small and superficial biopsy material and overlooked by a pathologist especially when lacking knowledge of this uncommon lesion. Verruciform xanthoma of the lower lip at the vermilion border is an extremely rare entity described in 5 cases worldwide to date. We aimed to bring verruciform xanthoma to readers' attention for a differential diagnosis of lower lip diseases and to prevent overdiagnosis and overtreatment of this lesion unfamiliar to otorhinolaryngologists. (C) 2017 The Author(s) Published by S. Karger AG, Basel.