Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 7 5 2023 02 12 234 237 Meisam Babaei Department of Pediatrics, North Khorasan University of Medical Sciences, Bojnurd, Iran. Susan Hossein Pardis Pathobiology and Genetics Laboratory, Mashhad, Iran. Maryam Tavakoli North Khorasan University of Medical Sciences, Bojnurd, Iran. Mahnaz Banaei Hazrat Ali Asghar Children’s Hospital, Iran University of Medical Sciences, Tehran, Iran. Maryam Bagheri Student Research Committee, North Khorasan University of Medical Sciences, Bojnurd, Iran. 2022 03 26 2022 09 07 In the fifth’s SARS-CoV-2 infection peak, although extended vaccination, still there is some reports of neurologic complications of covid-19 in children like adult. Though often we expose to benign neurologic features but sometimes there is unusual clinicoradiologic presentation. Thus, report of uncommon neurologic manifestations can help us to better understand of main pathophysiology in pediatric brain involvement.In this report, we present a five-year- old previously healthy girl that arrive to our hospital with fever, vomiting and loss of consciousness, on lumbar puncture there was mild protein elevation without poleocytosis and nasopharyngeal swap PCR for COVID-19 was positive. Brain imaging 10 hours after admission, revealed findings suggestive of acute necrotizing encephalopa thy (ANE). In spite of extensive brain involvement, after prompt initiation of high dose methylprednisolone and IV immunoglobulin, there was dramatic improvement after 3 months’ follow-up. To our knowledge, this is the first Iranian report and the second report on the w orld. https://crcp.tums.ac.ir/index.php/crcp/article/view/667