Isolated non-compaction of the ventricular myocardium (INVM), also known as left ventricular hypertrabeculation or spongy myocardium, belongs to the "unclassified" cardiomyopathies according to the World Health Organization. The main characteristic of this entity is a prominent trabeculation of the left ventricle with deep intertrabecular recesses communicating with the ventricular cavity. The pathomechanism of INVM is thought to be an arrest in cardiac myogenesis with persistence of embryonic myocardial morphology. The most frequent clinical manifestations include congestive heart failure, ventricular arrhythmias and systemic thromboembolic events. The therapy of INVM comprises standard medical therapy for heart failure.