Short QT syndrome is a rare clinical entity, described just a few decades ago. This, coupled with the small number of cases described in the literature, makes difficult to understand its pathophysiology and have a clear idea about the treatment to be performed. So far, several genetic alterations associated with short QT syndrome and increased risk of sudden death, some even related with other syndromes (particularly Brugada syndrome), have been described. A case of a patient with short QT syndrome in whom thanks to the implant of a defibrillator an episode of sudden death was aborted, is presented. To our knowledge, this is the first case reported in Colombia and opens the possibility of similar cases in the country.