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Introduction: There has been an upsurge in the reporting of cases of Left Ventricular Noncompaction (LVNC) cardiomyopathy in medical literature in the last 35 years due to advances in medical imaging.The condition was first described in 1926 and the first reported case by echocardiography was in 1984. The American Heart Association considers LVNC a primary cardiomyopathy of genetic origin, while the European Society of Cardiology and the World Health Organization grouped it as an unclassified cardiomyopathy. Its variability in terms of genetic profile, phenotypic expression, clinical presentation, and histopathological findings makes it somewhat a variant of other cardiomyopathies., Case Presentation: Patients with LVNC cardiomyopathy may not have any symptoms or may present with ventricular arrhythmias, heart failure, thromboembolism, or sudden death. LVNC cardiomyopathy diagnosis is typically made by echocardiography, although there are higher resolution cardiac imaging techniques. Management will depend on the patient's clinical presentation. Due to its genetic association, there is a need to screen living relatives once the diagnosis is made in an individual., Conclusion: The aim of this paper is to review current knowledge on this condition., (© Association of Resident Doctors, UCH, Ibadan.)