Background and Objective: We report on a 42-year-old man with Stage IIA non-Hodgkin's lymphoma who developed a severe mucocutaneous blistering eruption. His diagnosis, paraneoplastic pemphigus, was based on clinical, histological, and immunofluorescence findings and confirmed by immunoprecipitation. Despite maximal therapy with plasmapheresis, corticosteroids, and mycophenolate mofetil and the subsequent addition of cyclophosphamide and cyclosporine, the condition was fatal. Conclusion: This case illustrates the refractory nature of this disease and the inadequacy of existing therapies. Antcdents et Objectifs: Nous rapportons le cas d'un homme g de 42 ans ayant un lymphome non hodgkinien de stade IIa et qui a dvelopp une ruption mucocutane grave. Le diagnostic, pemphigus noplastique, a t bas sur les rsultats cliniques, histologiques et d'immunofluorescence, et confirm par immunoprecipitation. Malgr les traitements agressifs base de plasmaphrse, de corticostrodes et de mycophnolate moftile, et l'addition subsquente de cyclophosphamide et de cyclosporine, 1'affection tait mortelle. Conclusion: Ce cas illustre la nature rfractaire de la maladie et l'insuffisance des mdicaments existants.