AbstractThe evaluation of syncope occurring during exercise or occurring spontaneously in highly trained individuals presents a unique diagnostic challenge. It is of critical importance to exclude potential life-threatening disorders such as hypertrophic cardiomyopathy, long QT syndrome, right ventricular dysplasia, anomalous coronary artery distribution, valvular heart disease, myocarditis, or exercise-induced arrhythmia. This review is not directed towards identifying, treating, or determining athletic eligibility of individuals with such disorders. Rather, we endeavour to discuss the pathophysiology of exercise-induced neurocardiogenic syncope and to address the role of head upright tilt testing in evaluating syncope in athletic individuals in whom proper evaluation has excluded the presence of ischaemic heart disease or primary structural or electrical heart disease.