The congenital junctional ectopic tachycardia, is an unusual tachyarrhythmia, with early clinic manifestation and poor antiarrhythmic drugs response with a great infant mortality reaching rates of 35%. It deserves a special attention in its timely detection as well as in its appropriate handling with different modalities of pharmacological and nonpharmacological therapy. We reported two cases in which age of presentation of tachyarrhythmia was at three months and whose particularity was the good response to antiarrhythmic drugs; verapamil and later propafenone, used for the acute stages and a combination of propafenone plus propranolol initially for the chronic stage. Due to intolerance it was necessary to change the treatment after a year for sotalol and digital with good response. We review the literature about this topic.