Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a recognised cause of sudden cardiac death during exercise in young athletes. Competitive exercise is also known to accelerate progression of ARVC and exercise restriction is an important part of disease management. Regular endurance training can induce physiological changes detectable on electrocardiography and imaging which may overlap with pathological findings caused by ARVC, thus making differentiation of athlete’s heart from ARVC difficult in some cases. This review will discuss the changes of athlete’s heart, particularly as it affects the right ventricle, the diagnostic criteria for ARVC and how diagnostic accuracy is affected in athletes. A practical approach to this clinical problem is outlined.