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Abstract:  Paraneoplastic pemphigus (PNP) is seen most frequently in the setting of Castleman’s disease (CD) in childhood. We report herein a 10-year-old girl with PNP appearing a few weeks after resection of a recurrent CD. Despite improvement in skin and mucosal lesions with prednisolone and azathioprine, she had severe bronchiolitis obliterans and died from respiratory failure a few months later.