Exertional syncope has been suggested to correlate with a cardiac aetiology, particularly when occurring in mid-stride. The aim of the study is to evaluate the incidence of cardiac disease among children presenting with exertional syncope, determine the influence of timing within activity, and determine the utility of genetic testing and implantable event monitors in the evaluation of cardiac syncope. The patients ≤18 years old with exertional syncope who underwent exercise stress testing between 2008 and 2019 were retrospectively included. Patients were assessed to be in one of three groups: mid-exertion (mid-stride syncope), peri-exertion (syncope during activity but not moving), and post-exertion (within minutes of the activity). A total of 334 patients were included; 46 % were mid-exertion, 18 % were peri-exertion, and 36 % were post-exertion. Thirteen patients (3.8 %) were diagnosed with cardiac syncope; n = 9 (69 %) mid-exertion. Only mid-exertional syncope was significantly associated with a cardiac diagnosis (OR: 2.6). Cardiac diagnoses included inherited arrhythmia syndromes (n = 9), abnormal coronary origins (n = 2), and supraventricular tachycardia (n = 2). Only catecholaminergic polymorphic ventricular tachycardia (n = 5) was associated with mid-exertional syncope (OR: 1.4). The definitive diagnostic test was exercise testing (n = 8), echocardiogram (n = 2), genetic testing (n = 1), ambulatory monitor (n = 1), and EKG (n = 1). Mid-stride syncope was more likely to result in a cardiac diagnosis, and exercise testing is the most common definitive test as catecholaminergic polymorphic ventricular tachycardia was the primary aetiology of exertional syncope in our cohort. Implantable event monitors and genetic testing could be helpful in ruling out cardiac disease.