Assessment and management of cardiac rhythm disorders in athletes is particularly challenging. An accurate diagnosis and optimal risk-stratification are often limited because of substantial phenotypic overlap between pathological entities and adaptive cardiovascular responses that normally occur in athletes. An accurate diagnosis, however, is particularly important in this population, as 2 competing risks need to be cautiously balanced: the risk of under-diagnosis of an arrhythmogenic substrate that may trigger life-threatening events versus the risk of over-diagnosis that may result in an athlete’s improper disqualification. Accordingly, the management of arrhythmias in athletes may pose therapeutic dilemmas, and often differs substantially compared with the general population. In this review, we present the most frequently observed arrhythmias in athletes and briefly discuss their pathophysiologic substrate. We further propose diagnostic and therapeutic strategies based upon current guidelines, official recommendations, and emerging evidence from relevant clinical investigations. We focus particularly on disparities in current guidelines regarding the management of certain rhythm disorders, as these areas of uncertainty may reflect the challenging nature of these disorders and may indicate the need for individualized approaches in every-day clinical practice. A better understanding of the normal electrophysiological responses to chronic exercise, and of the pathophysiological basis and the true clinical significance of arrhythmias in athletes, may enhance decision-making, and may allow for management strategies which more prudently weigh the risk-to-benefit ratio of each approach.