Castleman's disease is a rare, benign, lymphoproliferative disorder of unknown cause. The disease can be occasionally associated with a paraneoplastic pemphigus (PNP), an autoimmune mucocutaneous disorder commonly seen in neoplasms of lymphocytic origin. Castleman's tumor has been found in only 10 % of PNP patients, so the diagnosis depends mainly on the pathological examination. For pathological examination, complete surgical excision biopsy of uni-centric disease remains best mode of treatment. We report a case of retrospectively diagnosed case of castlemanís disease with PNP after pathological examination.Keywords: Castleman's disease, paraneoplastic pemphigus (PNP), histopathological examination.