Sudden cardiac death (SCD) in athletes is a rare but tragic complication of a number of cardiovascular diseases. Inherited causes such as the structural and arrhythmogenic genetic heart conditions are often found or suspected to be the underlying cause of death at post mortem examination. Physical activity and intense exercise may trigger cardiac arrhythmias in individuals with these conditions leading to SCD. Prevention and treatment strategies include individual athlete management strategies, coupled with public health measures such as universal cardiopulmonary resuscitation (CPR) training and availability of automatic external defibrillators (AEDs) in public places, thereby preventing SCD in both athletes and the general population. Where an athlete is known to have a cardiac condition, some restrictions from participation may be prudent, however, new evidence is emerging that perhaps current restrictions are too strict and could be relaxed in some cases. An athlete-centred model of care is essential to ensure the clinical implications and athlete preferences are balanced providing the best outcome for all concerned.