Arrhythmogenic right ventricular dysplasia(ARVD)is an inherited heart muscle disease.Myocyte apoptosis and fibro-fatty scar tissue predisposes patients to malignant ventricular arrhythmias.Patients may present to variety of surgical procedures with diagnosed ARVD.Surgical insult,catecholamine surge and physiological disturbance can be hazardous on the vulnerable myocardium and may result in life-threatening ventricular tachycardia or sudden cardiac death in the perioperative period.Anaesthetists have particular role in perioperative management of this patient population,meticulous perioperative planning,close haemodynamic monitoring and maintenance of physiological stability throughout helps to avoid devastating perioperative loss.