Background: Isolated non-compaction of the ventricular myocardium (INVM) is characterized by prominent and excessive trabeculations in one or more ventricular wall segments, with deep intertrabecular recesses communicating directly with the ventricular cavities. Diagnosis of the disease is based on the 2-D echocardiographic features. Despite the fact that many cases of INVM have been reported in children and adults since the initial description of the disease, it has been diagnosed in only a few fetuses up to now. Therefore, failure to diagnose on fetal echocardiography is probably more common. There are different opinions about diagnosis of INVM on fetal echocardiography.