Left ventricular noncompaction, or noncompaction cardiomyopathy, is a rare congenital cardiomyopathy caused by the failure of the myocardial compaction. It has distinct morphological characteristics in the ventricular cavity and main cclinical manifestations are heart failure, arrhythmia, and thromboembolic complications. This paper reports on a patient who was admitted to the hospital with a diagnosis of acute myocardial infarction and severe left ventricular systolic dysfunction. During the patient's assessment and investigation, left ventricular noncompaction cardiomyopathy was diagnosed. In this literature review, we discuss the diagnostic criteria and the treatment of these patients.