Abstract
Proliferative verrucous leukoplakia (PVL) is a distinct progressive and multi-focal form of oral leukoplakia, not associated with the traditional risk factors (ie, tobacco and alcohol consumption). The incidence of oral squamous cell carcinoma in PVL patients is high. Here, we describe the case of a patient affected by PVL, who developed two metachronous oral verrucous carcinomas at different sites of the oral mucosa. Owing to the high risk of multiple oral squamous cell carcinoma, periodical clinical and histopathological follow-up is mandatory and should continue lifelong.