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Perioperative management of an infant with intracranial tumor and congenital cyanotic heart disease poses a lot of challenges to the neuroanesthesiologist and neurointensivist. Maintaining both the cardiovascular and cerebrovascular homeostasis are of paramount importance for a successful outcome. In this case report, we have mentioned the perioperative challenges of a 7-month-old baby with tetralogy of Fallot who underwent craniotomy and excision of choroid plexus papilloma.