Malignant hyperthermia (MH) is an acute hypermetabolic crisis triggered in susceptible patients by the administration of succinylcholine or a volatile anesthetic agent. When providing anesthetic care for MH-susceptible agents, a total intravenous anesthetic (TIVA) technique is frequently chosen. When choosing the components for TIVA, several options exist including the combination of propofol or dexmedetomidine with an opioid. We present our experience with the use of dexmedetomidine as a key component of the anesthetic regimen in a 5-month-old infant with a family history of MH. Previous reports of the use of dexmedetomidine in MH-susceptible patients are reviewed and its benefits in such patients discussed.