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Paraneoplastic pemphigus (PNP) is a mucocutaneous disease that occurs in association with an underlying neoplasm. Oral mucosal lesions may be the only manifestation of this condition, or they may be observed in association with cutaneous lesions. The prognosis of PNP is generally poor, and the disease is often fatal. This article highlights an aggressive case of PNP that initially presented with oral mucosal lesions and emphasizes the importance of a multidisciplinary approach for evaluation and management of this condition.