AbstractThe anomalous origin of a coronary artery (AOCA) is a challenging topic, due to its rarity, the complexity of the pathophysiological aspects, the clinical presentation (often silent), the difficulty of diagnosis, and the potential risk of causing acute cardiovascular events up to sudden cardiac death, particularly when triggered by heavy physical exercise or sport practice. Increasing interest in sport medical literature is being given to this topic. This paper reviews current knowledge of AOCAs in the specific context of the athletic setting addressing epidemiological and pathophysiological aspects, diagnostic work‐up, sports participation, individual risk assessment, therapeutic options, and return to play decision after surgery.