Proliferative verrucous leukoplakia (PVL) is a rare aggressive subset of oral premalignancy with preponderance in women and lack of risk factors. A 65-year-old man who smoked for 40 years was referred with clinical suspicion of oral squamous cell carcinoma. On clinical examination, multiple painless erythroleukoplakia throughout the tongue and hard palate were observed. Oral lesions were not removable by scraping and had a long period of evolution. After biopsy, the microscopic examination revealed mild epithelial dysplasia on the palate and severe on the tongue. Based on the progressive clinical course of the oral lesions, a diagnosis of PVL was made. This case highlights the close communication of the pathologist and clinician in determining the diagnosis of PVL and progressive clinical course of the disease.