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Catecholaminergic polymorphic ventricular tachycardia is a rare cause of exercise-induced arrhythmia and sudden cardiac death in the pediatric patient. This arrhythmia is difficult to diagnose in the emergency department, given the range of presentations; thus, a familiarity with and high index of suspicion for this pathology are crucial. Furthermore, recognition of the characteristic electrocardiogram findings and knowledge of the management of the symptomatic patient are necessary, given the risk of arrhythmia recurrence and cardiac arrest. In this review, we discuss the presentation, differential diagnosis, and management of catecholaminergic polymorphic ventricular tachycardia for the emergency care provider.