Isolated left ventricular non-compaction (LVNC) is a rare congenital disorder caused by failure of normal embryogenesis of the endocardium and myocardium in utero, resulting in prominent ventricular trabeculations with deep intertrabecular recesses. Patients may be asymptomatic or may present with refractory cardiac failure. The best modality of diagnosis is based on echocardiographic diagnostic criteria. Prognosis remains poor for patients with impaired systolic left ventricular function, as treatment options are very limited. We report the case of 2-month-old infant presenting with congestive cardiac failure diagnosed as isolated LVNC on echocardiography.