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© Korean Medical Association This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons. org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. An irregular heart rhythm is a common concern among children. Recognizing the cause of the irregular rhythm is crucial for the proper diagnosis and management by primary physicians as well as pediatric specialists. The cause of pediatric arrhythmias can be identified based on the clinical history, physical examination, presenting symptoms, and electrocardiogram. Pediatric arrhythmias are classified as benign and non-benign. Both benign and non-benign arrhythmias can originate from the sinoatrial node, atrial myocardium, atrioventricular node, and ventricle myocardium. The common form of benign arrhythmias, including sinus arrhythmia, premature atrial contraction, premature ventricular contraction, and Wenckebach atrioventricular block, have no clinical significance or need for therapy. On the other hand, non-benign arrhythmias, such as supraventricular tachycardia, ventricular tachycardia, high-degree atrioventricular conduction abnormalities, and genetic arrhythmia, can affect the hemodynamic state and may cause a serious condition in pediatric patients. Most cases of benign arrhythmia are asymptomatic, and the prognosis is favorable for the normal heart. In this article, we review our current understanding of the electrocardiographic characteristics, clinical presentation, etiology, and natural history of benign arrhythmias in pediatric patients.