Professional and amateur athletic training can cause tremendous overload of the cardiovascular system and thus become a trigger of serious and often fatal cardiac events in athletes with a previously undetected underlying cardiovascular disease. Therefore, every athlete should undergo a specialized diagnostic and qualification screening before a training program is prescribed or continued. However, it is still an unresolved issue which of the accessible diagnostic tools should be routinely applied in order to increase the safety of extreme physical training and reduce the risk of sudden cardiac death. Pre-participation athlete evaluation including a standard electrocardiogram (ECG), physical examination, and familial history of cardiovascular diseases is important, but does not always guarantee high diagnostic accuracy. Hence, the aim of this review article is to discuss the controversy over the usefulness of ECG for the detection of cardiovascular diseases in athletes as well as the views on athlete screening methods in Europe and the USA. Differential diagnostic options and screening schemes are also described in particular groups of athletes in reference to their age, cardiovascular risk factors, as well as intensity and type of sport discipline.