Castleman's disease is a rare lymphoproliferative disease which can mimic other malignant tumours and can be associated with paraneoplastic pemphigus especially in the paediatric age group. This presentation can be challenging even to the seasoned anaesthesiologist when it comes to managing such a case presenting for laparotomy. Case presentation: Here such a case in a 12-year-old who was initially diagnosed as retroperitoneal sarcoma complicated with paraneoplastic pemphigus and inferior

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... val thrombus is presented and its anaesthetic management and challenges are discussed. Special care needs to be taken while anaesthetizing such a patient keeping in mind the difficult airway, adrenocortical suppression, and exacerbation of skin lesions due to various anaesthetic procedures. For this case, the histopathology turned out to be Castleman's disease. Conclusion: The anaesthetic challenges associated with paraneoplastic pemphigus in paediatric age group receiving perioperative thromboprophylaxis need special mention. Here we are discussing the challenges and anaesthetic management of such a case as literature on this aspect is few.