Noncompaction of the ventricular myocardium is a rare congenital disorder 
characterized by the presence of numerous prominent trabeculations and deep 
intertrabecular recessess which communicate with the left ventricular cavity. 
The disease uniformly affects the left ventricle, and sometimes also affects the 
right ventricle. Echocardiographic findings are important clues for the 
diagnosis. Clinical symptoms include signs of left ventricular systolic 
dysfunction even to the point of heart failure, ventricular arrhythmias, and 
embolic events. We describe an illustrative case of isolated noncompaction of 
the left ventricular myocardium in a two-year-old child with the typical 
clinical and echocardiographic features of the disease. The literature on the 
topic is reviewed.