A 72-year-old former smoker was diagnosed with Extramammary Paget disease (EMPD) of the oral mucosa, a condition rarely seen outside breast tissue. Initially mistaken for lichen planus, a biopsy confirmed EMPD, making it only the ninth documented case in 50 years. Cleveland Clinic’s multidisciplinary tumor board led the treatment, with Dr. Danielle Bottalico performing a wide local excision and mandibulectomy, followed by Dr. Jamie Ku’s reconstructive surgery using forearm tissue. The patient recovered well, required no adjuvant therapy, and remains disease-free 18 months post-surgery. Given EMPD’s rarity and potential association with invasive adenocarcinoma, early biopsy and expert pathology review are crucial. The team’s findings were published in Head and Neck Pathology.