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Background: Seafarers are at an increased risk of developing cardiovascular diseases (CVDs), potentially due to a stressful working environment and behavioral risk factors. To develop better prevention strategies, it is important to elucidate the extent of this risk. Therefore, we conducted a systematic literature review on CVD in seafarers. Method: We conducted systematic searches in five databases. All studies investigating CVDs among occupational seafarers, published in articles or conference papers, were eligible for inclusion. The identified records were screened and reviewed by two independent researchers, who also evaluated the methodological quality of the included studies. Results: Three thousand nine hundred and seventeen records qualified for screening, and 55 were eligible for inclusion. Most of the studies were observational, including cohort, frequency, incidence or prevalence studies, and review of case records. Around half were assessed at risk of biased findings. Participants in the studies were primarily from North America or the European continent and work onboard transportation vessels. Many studies investigated CVDs as a cause of death, focusing on conditions such as CVD, ischemic heart disease, and myocardial infarction. Frequency of CVD conditions varied but indicate that seafarers face a greater risk compared to the reference populations or control groups. Environmental factors were mainly investigated as risk factors. Conclusion: Our results indicate a higher risk of CVDs among seafarers compared to reference or control groups. However, due to the variable quality of the evidence, well‐designed studies are needed to establish the causes of cardiovascular mortality and morbidity in seafarers and to investigate behavioral aspects of cardiovascular risk. [ABSTRACT FROM AUTHOR]