Tehran University of Medical Sciences Archives of Anesthesiology and Critical Care 2423-5849 11 4 2025 04 05 606 609 Sogol Asgari Anesthesiology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Seyed Sam Mehdi Hosseininasab Anesthesiology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Faranak Behnaz Department of Anesthesiology, School of Medicine, Shohada-e-Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. 2024 12 05 2024 12 23 There is a rare genetic disorder called Jervell-Lange Nielsen syndrome that leaves people congenitally deaf and with a long QT interval. This can lead to deadly heart rhythm problems and sudden death. For the treatment of hearing loss, cochlear implants, and for the treatment of heart difficulties, beta-blockers, and in certain circumstances, implantable cardioverter defibrillators, arrhythmias, syncope attacks, and sudden death are recommended. We discuss the case of an 8-year-old child who was referred for cochlear implantation after being diagnosed with Jervell-Lange Nielsen syndrome. In this study, we want to deal with patient management preoperatively and during surgery and describe the side effect of this syndrome. https://aacc.tums.ac.ir/index.php/aacc/article/view/1152