Paraneoplastic pemphigus (PNP) is a dermatosis with 90% mortality which frequently develops secondary to lymphoproliferative malignancies. Non-Hodgkin lymphoma and chronic lymphocytic leukemia (CLL) are the mostw common accompaniying lymphoproliferative disorders in PNP. PNP can be part of the clinical variant of paraneoplastic autoimmune multiorgan syndrome (PAMS). In this case, internal organs, especially lung and kidney may expose to autoimmune attack and subsequently organ failure may develop. Bronchiolitis obliterans is a main cause of death in patient with PAMS due to acute lung failure. Here we report, a case of paraneoplastic pemphigus in which polymorphic lesions developed following the diagnosis of CLL in a 58-year-old male patient. Polymorphic lesions as well as underlying malignancy as a two major criteria and IgG, C3 deposition in direct immunofluorescence examination and acantholysis as a two minor criteria determined by Camisa and Helm allowed us to make a diagnosis PNP. The main cause of the death in PNP is a sepsis secondarily to immunosuppression. We lost this patient due to sepsis after methylprednisolone and intravenous immunoglobulin therapy.