BACKGROUND Myocardial infarction (MI) is one of the most serious conditions presenting to the Emergency Department. Typical/classical symptoms of MI include chest pain and tightness that is referred to the left arm. CASE REPORT We present a case of ST-elevation myocardial infarction (STEMI) with a primary atypical presentation of right ear pain. The patient was diagnosed with STEMI based on electrocardiogram (ECG) and cardiac markers. As a result, the patient underwent percutaneous intervention (PCI), which showed an occluded left anterior descending artery (LAD) compared to the previous reported studies with similar presentation in which the right coronary artery (RCA) was affected. CONCLUSIONS While MI is known for its typical presentation, being vigilant to patients who present atypically especially when they possess risk factors is extremely important and doing so can be critical to the outcome because MI treatment is dependent on fast and prompt management.