According to the World Health Organization classification of cardiomyopathies, left ventricular noncompaction is still an unclassified cardiomyopathy. In 2006, the American Heart Association classified this entity as a primary cardiomyopathy of genetic origin. In 2008, the European Society of Cardiology updated the classification scheme similar to the World Health Organization classification. At present, there is no consensus on the diagnostic criteria, and diagnosis is based on the morphologic features identified by cardiac imaging studies or at autopsy. Due to lack of standardization of the diagnostic criteria and little awareness of this condition among clinicians, the true prevalence of this disease is not clear. There is no specific therapy for this condition. However, it seems prognosis is much better than initially reported. The current status of diagnosis, prognosis, and management of isolated noncompaction in adults is discussed in this review.