Rationale:Proliferative verrucous leukoplakia (PVL) is a multifocal, slowly evolving lesion that resists all types of treatment and has a high propensity for malignant transformation into oral squamous cell carcinoma. Lack of awareness and acquaintance with white lesions of the oral cavity makes it difficult to diagnose. Besides being rare, PVL significantly aggressive, so clinicians need to be aware of it carefully. Therefore, it is recommended to have the earliest possible diagnosis and total excision of this lesion. We report this case to present typical clinical and histologic features of PVL so a For the purpose of sensitizing clinician.Patient concern:A 61-year-old female came to the clinic concerning of recurring painless, white patch on the tongue 2 months ago, associated with oropharyngeal dryness.Diagnoses:This case satisfies these major and minor criteria to diagnosed PVL.Intervention:An excisional biopsy of the lesion was done to check for the presence of dysplasia, as lesions were persisting. Hemostasis was achieved with single interrupted sutures.Outcome:no recurrence has been observed since excisional 1 year follow-up.Lesson:The key feature is early detection, precisely in cases of PVL it is critical for better treatment outcomes, lifesaving, quality-of-life enhancement. To detect and treat any potential pathologies, clinicians should meticulously examine the oral cavity and patients have to be aware and informed of the importance of regular screenings. This lesion is resistant to the presently available treatment modalities; therefore, total excision with free surgical margins is critical combined with a lifelong follow-up.