Proliferative verrucous leukoplakia (PVL) is a rare less recognized form of oral leukoplakia. Patients with this condition represent a unique clinically and pathologically progressive characteristic from conventional leukoplakia. Because of the lack of defined pathologic lesions, identifying patients with the early diagnosis of PVL is challenging. This is largely due to the overlapping clinical and pathologic early features with conventional multifocal leukoplakia with dysplasia. The diagnosis can only be achieved through the keen clinical observation of the temporal progression in individual patients to verrucous and/or conventional squamous carcinoma. We present a brief view of the clinicopathologic and biological characteristics of PVL and discuss diagnosis, differential diagnosis, and management.