Cardiovascular disease (CVD) is the leading cause of mortality worldwide. An unhealthy diet, among other risk factors, can be involved in the development of the disease. This type of diet is usually characterized by being low in unprocessed foods (fruits, vegetables, etc.) and high in ultra-processed foods (UPFs). It has been suggested that a high consumption of UPFs could be related to the development of CVD. However, as UPFs are a broad group in which multiple foods with different compositions coexist, it is not only difficult but also a generalization to draw a conclusion about their possible association. The present systematic review studies the relationship between the consumption of UPFs and the risk of CVD. A systematic review was performed in the PubMed database. PRISMA criteria were applied to identify articles that met the following criteria: studies performed in humans, in English or Spanish, and be original articles, excluding meta-analyses and reviews. The search was performed using the terms “Ultra-processed food” or “Fast food” and “Cardiovascular disease” or “Cardiovascular risk factor”, with a total of 10 articles included: 4 prospective cohort studies, 4 cross-sectional studies and 2 modeling studies. An association between the consumption of UPFs and CVD was found in all the studies analyzed. However, there are limitations with respect to the use of different food classification systems according to the degree of processing. This systematic review concludes that more studies are needed to establish with greater evidence the relationship between the consumption of UPFs and risk of CVD.