Paraneoplastic Pemphigus is a rare autoimmune disease associated primarily with lymphoproliferative diseases, (benign or malignant). It is characterized clinically by numerous, chronic persistent and painful oral ulcerations, together with various skin eruptions like Erythema multiformis; histologically by the presence of subepithelial bullae and cleft formation and immunologically by the detection of autoabs against intracellular desmosomal and hemidesmosomal plaque proteins (envo, peri, desmo-plakin and alpha 2macrobulin like Ag). Early detection and treatment (i.e surgical removal) of the primary tumor together with i.v administration of immunoglobulins is the treatment of choice for this lethal bullous disease.