Abstract
Acute necrotizing encephalopathy (ANE) is a progressive neurodegenerative condition that often arises after exposure to a variety of viruses and systemic infections, including influenza and SARS-CoV-2. It typically presents with developmental delay, seizures, dysarthria, and ataxia. Acute necrotizing encephalopathy type 1 (ANE1) is recurrent, and familial cases have been associated with mutations in the RAN-binding protein 2 (RanBP2) gene. The disease shares symptomatic and pathological resemblance with mitochondrial metabolic disorders. In this case report, we present the anesthetic management of a seven-year-old boy with ANE1 who underwent total hip reconstruction. Literature on the anesthetic management of such patients is sparse, and we discuss the patient's perioperative anesthetic management, including medications, monitoring, and care, along with a literature review.