Paraneoplastic pemphigus (PNP) is a recently recognized autoimmune mucocutaneous disease associated with a few unusual lymphoreticular neoplasms. Intractable stomatitis is the most constant clinical feature but the cutaneous presentations are characteristically variable. We describe a patient with PNP associated with chronic lymphocytic leukemia who presented with severe oral, laryngeal, and pharyngeal involvement. The only cutaneous finding was a pemphigus vegetans-like plaque, a previously undescribed manifestation. The plaque evolved from a flaccid blister that formed at a prior intravenous catheter site. The mucosal biopsy specimen demonstrated faint intercellular staining with IgG, whereas results for the cutaneous plaque were negative. Indirect immunofluorescence testing was positive on both monkey esophagus and rat bladder epithelium. Immunoprecipitation (IP) studies demonstrated a complex of 4 proteins with molecular weights of 250, 230, 210, and 190 kd, confirming the diagnosis of PNP. (J Am Acad Dermatol 1998;39:867-71.)