Left ventricular non-compaction is a form of cardiomyopathy defined by a spongy left ventricular myocardium. Despite the condition being the leading reason for referral for patients with depressed left ventricular ejection fraction, current classification systems categorize it as an unclassified cardiomyopathy. The lack of recognition as a distinct form of cardiomyopathy has undermined research and expert consensus on diagnosis and specific clinical management guidelines. In addition, current research on epidemiology, diagnosis and clinical management are small scale-studies with inconsistent findings. The purpose of the present review is to combine research findings on pathogenesis, prognosis, clinical manifestation, diagnosis and clinical management methods. The intention is to advance knowledge and understanding of the clinical status and management of left ventricular non-compaction.