Non-compaction of the left ventricle (NCLV) was categorised as unclassified cardiomyopathy by the World Health Organization in 1995. Over the last decade this condition has been identified as a distinct form of cardiomyopathy and a genetically heterogeneous disorder. Clinically, this may be coupled with the loss of contractility, arrhythmia, and thromboembolism. The prognosis in a symptomatic patient is generally poor, with progression to chronic heart failure and death, including sudden death. We report a case of a child with NCLV and coexisting hemodynamic significant ventricular septal defect.