We present the case of a 71-year-old white male with paraneoplastic pemphigus associated with a B-cell non-Hodgkin's lymphoma. Diagnosis of paraneoplastic pemphigus was made by the characteristic findings on immunoprecipitation performed on a serum specimen. Paraneoplastic pemphigus is a severe autoimmune disease comprised of polymorphous mucocutaneous lesions, characteristic laboratory findings, association with one of several types of neoplasms, and a very poor prognosis.