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Isolated noncompaction cardiomyopathy is an exceedingly rare congenital cardiomyopathy. A case of isolated noncompaction cardiomyopathy is reported and the literature on the subject collected through a comprehensive literature search is reviewed. Fewer than 100 cases of this condition have been reported. Isolated noncompaction cardiomyopathy is caused by a defect in cardiac morphogenesis resulting in an arrest of compaction of loose interwoven meshwork of myocardial fibers during intrauterine life, which results in severe systolic dysfunction as well as undue hypertrophy of the involved walls of the ventricles. Although the most frequent sites involved are left ventricular apex and inferior wall, involvement of other left ventricular walls and right ventricle has also been reported. Etiology of the isolated noncompaction of myocardium is not clear. Familial cases have been reported and the mode of inheritance is heterogeneous. In X-linked form of the disease, a locus has been found on Xq28, and mutations have been reported in G4.5 gene. The age of onset of symptoms ranges from infancy to the geriatric age. Patients with isolated noncompaction cardiomyopathy have a high incidence of heart failure, arrhythmias, and thromboembolism. The most common presentation is congestive heart failure. Arrhythmias include atrial arrhythmias, ventricular tachycardia, and sudden cardiac death. The patient reported in this article presented with paroxysmal supraventricular tachycardia. Echocardiography is the procedure of choice to establish diagnosis. Due to the lack of associated cardiac anomalies, antenatal detection is difficult. The treatment is that for congestive heart failure, arrhythmias, and thromboembolism. The end-stage congestive heart failure is managed with heart transplantation and potential life-threatening ventricular tachyarrhythmias with an implantable cardioverter defibrillator. Prognosis is poor and the common causes of death are intractable heart failure and sudden cardiac death.