Left Ventricular Non-Compaction (LVNC) is a rare congenital cardiomyopathy that is characterised by thin compacted epicardial layer and extensive non-compacted endocardial layer with prominent trabeculations and deep recesses. It may vary from mild to severe forms and may present at any age. It has a strong hereditary and genetic predisposition and variable penetrance. It is manifested as CHF, arrhythmia or DCM. It can be diagnosed by Echocardiography, although MRI is superior modality. The treatment includes beta-blockers, antiarrythmics, decongestive drugs, ICD and anticoagulants. Most of LVNC patients (severe forms) need heart transplantation.