This paper focuses on a rare but aggressive form of leukoplakia with malignant potential and is an important reminder of the need for specialist management to those in allied specialties such as ENT that may initially be referred these oral lesions. Proliferative verrucous leukoplakia (PVL) manifests as progressive, multifocal, exophytic lesions that are persistent, irreversible, and more common in elderly women, currently without any known risk factors. Management cannot be determined on initial histology alone, and requires monitoring of temporal clinical changes in addition. This UK based tertiary centre used a retrospective review of all PVL cases in their unit over a 25-year period to determine how their more conservative approach compared with published outcomes. Forty-eight patients were identified. Eight percent of premalignant lesions underwent surgical excision (compared with 80% in a recent systematic review of 126 cases), the remainder were managed conservatively. Almost 50% underwent malignant transformation and six patients died from their disease. This compares similarly with published rates of transformation that were managed more aggressively with surgery at the premalignant stage. The median follow-up in the transformed group was 8.2 years, and 3.5 years in those who did not. The authors admit that the shorter follow-up in their cohort may have underestimated mortality rates. The take-home message of this paper is the need for careful and lifelong surveillance of patients with PVL in the context of a specialist unit, with a low threshold for repeat biopsy to detect malignant transformation.