This review highlights and discusses recent advances in understanding left ventricular non - compaction (LVNC). Clinical profile, prognosis and even diagnosis are still a great challenge faced by the world. The population prevalence of left ventricular non - compaction remains unknown. High variability of clinical manifestations, genetic heterogenity with overlap of different phenotypes, variability of hereditary patterns suggests that LVNC seems to be rather an isolated trait or a part of phenotypic expression of different cardiac diseases or complex genetic syndromes.