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.