T HE RECENTLY RECOGNIZED MYOCARDIAL malformation known as ventricular noncompaction is a fascinating disorder, characterized by the presence of an extensive trabeculated myocardial layer reinforcing the luminal aspect of the compact portion of the ventricular wall. Usually described thus far in the left ventricle, it is typically associated with abnormal systolic function, albeit also recorded with restrictive physiology and segmental hypokinesis. 1-5 Seen most frequently in the structurally normal heart, increasing recognition shows that the lesion can also accompany diverse forms of congenital cardiac disease (Figs 1 and 2). 1-10 When seen in the left ventricle, it is currently considered an unclassified form of cardiomyopathy, being neither dilated, hypertrophic, nor restrictive. 2,3,11,12 Although once considered rare, 13-15 it is now being seen with increasing frequency, from the fetus to the adult. 16-29 Diagnosis has now moved from the autopsy table to recognition during life, albeit the debate continues with regard to the features displayed by angiography, echocardiography, computed tomography, and magnetic resonance imaging that permit unequivocal recognition. In this review, we will consider myocardial noncompaction from the stance of comparative vertebrate biology, as well as discussing the genetic, developmental, clinical, and imaging characteristics of patients with noncompaction, seen both in isolation and in the setting of congenital cardiac disease. In the latter setting, we must ask whether noncompaction is a homogeneous process that, in its many clinical and pathological expressions, can be explained on the basis of a developmental abnormality. In this respect, it is of interest to note that, in the evolution of the vertebrates, myocardial noncompaction is advantageous, and indeed necessary, for the circulatory function of some