ST segment elevation acute coronary syndrome is a clinical condition that is rarely observed in pregnant women. However, its manifestation is a situation of high maternal-fetal risk. Pharmacotherapeutical management of these patients is difficult and requires individualized care by a multidisciplinary team since many of the standard treatments are included within the categories of teratogencity C or D of the Food and Drug Administration and experience with techniques such as coronary angioplasty with stent placement is scarce. The case of a 32-year woman who was 11 weeks pregnant and diagnosed of acute coronary syndrome with ST segment and its therapeutic approach are described. Furthermore, the information available on epidemiology, etiology and pathophysiology of acute coronary syndrome with ST segment during pregnancy and the specific role of the currently available treatment options are reviewed.