Athletes enjoy excellent health outcomes including greater longevity relative to non-athletic counterparts. Paradoxically, however, endurance athletic conditioning is associated with an increase in some arrhythmias. This review discusses the potential mechanisms for this paradox and strategies enabling early identification of potentially serious pathologies. Screening remains contentious due to the challenges of identifying relatively rare entities amongst a healthy cohort. The imperfect diagnostic accuracy of all current tests means that screening strategies have potential for harm through incorrect diagnoses as well as the potential for identification of important sub-clinical pathologies. Management of athletes at risk of ventricular arrhythmias and sudden cardiac death is similarly complex. There is much yet to learn about the specific patterns of ventricular arrhythmias in athletes, and the separation of benign from potentially life-threatening remains imperfect. There are some promising advances, however, such as specialised imaging modalities combined with improved electrophysiological diagnostics and therapeutics. Some unique clinical patterns are emerging to advance our understanding and management of athletes with ventricular arrhythmias, requiring specialised skillsets for evaluation and management.