Short QT syndrome (SQTS) is a gene-related arrhythmogenic syndrome harbouring a large spectrum of symptoms ranging from mild palpitations to sudden cardiac death.The mutation of genes (KCNH2, KCNQ1, and KCNJ2) encoding for cardiac potassium channels plays a central role in SQTS. Electrocardiography is the primary important step in the diagnosis (short QT interval along with T wave changes), but ECG findings may be easily ignored. Treatment of the syndrome is still controversial. Some specific antiarrhythmic drugs and an implantable converter/defibrillator (ICD) have been considered as main therapeutic strategies. ICD implantation may be a life-saving procedure due to the presence of sudden cardiac death risk in patients with SQTS, but ICD-related problems such as inappropriate shock deliveries due to oversensing of prominent T waves have made medical therapy an alternative option. Notwithstanding the scarcity of cases, clinicians should keep this syndrome in mind, and be familiar with its clinical findings particularly when evaluating patients with palpitation, syncope or a history of sudden cardiac death.We present a brief review of the literature concerning the aetiology, clinical findings and therapeutic approach to this rare entity.