A 33-year-old man with recessive dystrophic epidermolysis bullosa presented with a 3-month history of an enlarging mass within scarring on the posterior aspect of the right shoulder. The clinical appearance of the mass with an almost cobbled, verrucous surface, and its rapid evolution suggested the development of a squamous cell carcinoma (SCC) in a chronically scarred site. Histopathological examination of a biopsy taken from the lesion subsequently revealed it to be a verruciform xanthoma. This case shows that benign phenomena can mimic SCC and underlines the need for a biopsy to be taken promptly.