Patients with systemic lupus erythematosus (SLE) are at increased risk of coronary heart disease (CHD). Even though the absolute risk of cardiovascular disease (CVD) among SLE patients increases with advancing age, younger female patients are at the greatest risk of developing acute myocardial infarction (AMI). These young patients are not considered to be at high risk for CVD using traditional risk assessment tools. Also, subclinical atherosclerosis is less common among young lupus patients. AMI could present with or without significant obstruction in coronary arteries in younger patients. There are no guidelines on appropriate cardiac screening of younger lupus patients, often without chest pain or who present with non-specific complaints. In recent years, the incidence of acute coronary syndrome (ACS) and ST-segment elevation AMI has decreased in the general population and in older lupus patients. Why has a similar decline in cardiovascular (CV) events not been seen in younger lupus patients? Since the issue of CVD in younger lupus patients is under-researched, a narrative review, rather than a systematic literature review was performed, based on the selected articles and points of view relevant to the topic. The aim of this review is to raise awareness of the relationship between SLE and CVD in younger ages, discuss possible non-atherosclerotic mechanisms of obstructive and non-obstructive CHD in lupus, elaborate on acute coronary syndromes unique for young patients, point out current challenges in identifiing at-risk patients for ACS, potential for new imaging techniques, the need for individualised treatment, with or without coronary stenting in ACS, and to underscore the relevance of CVD studies in young patients with SLE.