Noncompaction cardiomyopathy is a condition characterized by the persistence of embryonic structures in the heart muscle. In the definition and classification of cardiomyopathies drawn up by the World Health Organization in 1995, this anomaly is counted among the “unclassified” cardiopathies. From a genetic point of view, it follows the mutation of one of at least 14 genes, many of which are also responsible for other heart diseases (hypertrophic, dilated, and Brugada syndrome). The most important genes are SCN5A, MYH6, and LDB3. About two thirds of patients with noncompaction cardiomyopathy have signs and/or symptoms of heart failure, which can occur from the first months of childhood or start later. Some forms of noncompaction cardiomyopathy are known to fall under the clinical signs of muscular dystrophies (including Duchenne, Becker and Emery Dreifuss). Early diagnosis is essential to prevent possible complications that can lead to sudden death.