Junctional ectopic tachycardia (JET) is a rare supraventricular tachycardia more frequently seen in pediatric patients which is associated with high morbidity and mortality without an adequate treatment. Its treatment often requires multiple drugs for arrhythmia control and, in some cases, a cryoablation is indicated. Some patients may revert to sinus rhythm spontaneously after long periods on antiarrhythmic drug. Currently, the arrhythmia prognosis has improved significantly since cryoablation development and the implementation of a more aggressive pharmacological treatment strategy. The purpose of this review is to describe the natural history of idiopathic JET and treatment advances.