Left ventricular non-compaction (LVNC) is a rare cardiomyopathy, which is today, due to modern ultrasound technology more frequently detected in clinical practice. It is caused by the failure of normal embryonic development of the myocardium from loosely arranged muscle fibers to the mature compacted form of myocardium. Morphologic presentation consists of unique two-layered structure, a thick noncompacted endocardial and a thin compact epicardial layer, in infero-lateral and apical segments. The endocardial layer contains loosely arranged muscle fibers, prominent trabeculations and deep perfused intertrabecular recesses. It could be diagnosed both as an infantile or adult type, appearing sporadically or among families where it is transferred x-linked or autosomal dominant. The recognition of the disease is mandatory because of its high mortality and morbidity due to the progressive heart failure, thromboembolic events and lethal arrhythmias. Echocardiography is the procedure of choice to confirm the diagnosis and in the follow-up of patients with LVNC. A literature review about LVNC pathogenesis, diagnostis, and treatment was discussed.