Globally, cardiovascular disease remains a major cause of adverse outcomes in young individuals, unlike its decline in other age groups. This group is not well studied and has a unique risk profile with less traditional cardiovascular risk factors compared with older populations. Plaque rupture still remains the most common etiology of myocardial infarction, but unique syndromes such as plaque erosion, coronary microvascular dysfunction, spontaneous coronary artery dissection, and coronary spasm related to drug use are more prevalent in this age group. Such diversity of diagnosis and presentation, along with therapeutic implications, underscore the need to study the profile of myocardial infarction in young persons. We searched PubMed for articles published from 1980 to 218 using the terms acute myocardial infarction, young, plaque rupture, plaque erosion, spontaneous coronary artery dissection (SCAD), coronary vasospasm, variant or Prinzmetal angina, drug-induced myocardial infarction, myocarditis, coronary embolism, microvascular dysfunction, MINOCA, and myocardial infarction in pregnancy and reviewed all the published studies. With the data from this search, we aim to inform readers of the prevalence, risk factors, presentation, and management of acute myocardial infarction in young patients and elaborate on special subgroups with diagnostic and therapeutic challenges. We also outline a parsimonious method designed to simplify management of these complex patients.