BackgroundSudden cardiac death (SCD) is the leading medical cause of death in athletes. To prevent SCD, screening for high‐risk cardiovascular conditions (HRCC) is recommended. Screening strategies are based on a limited number of studies and expert consensus. However, evidence and efficacy of athlete HRCC screening is unclear.ObjectiveTo determine methodological quality and quality of evidence of athlete screening, and screening efficacy to detect HRCC in a systematic review.MethodsWe performed a systematic search of Medline, Embase, Scopus and Cochrane Library up to June 2021. We included articles containing original data of athlete cardiovascular screening, providing details of screening strategies, test results and HRCC detection. We assessed methodological quality of the included articles by QUADAS‐2, quality of evidence of athlete HRCC screening by GRADE, and athlete HRCC screening efficacy by SWiM.ResultsOf 2720 citations, we included 33 articles (1991–2018), comprising 82 417 athletes (26.7% elite, 73.4% competitive, 21.7% women, 75.2% aged ≤35). Methodological quality was ‘very low’ (33 articles), caused by absence of data blinding and inappropriate statistical analysis. Quality of evidence was ‘very low’ (33 articles), due to observational designs and population heterogeneity. Screening efficacy could not be reliably established. The prevalence of HRCC was 0.43% with false positive rate (FPR) 13.0%.ConclusionsMethodological quality and quality of evidence on athlete screening are suboptimal. Efficacy could not be reliably established. The prevalence of screen detected HRCC was very low and FPR high. Given the limitations of the evidence, individual recommendations need to be prudent.