Dilated cardiomyopathy (DCM) is the most prevalent form of cardiomyopathy, and sudden cardiac death (SCD) remains a common event in young patients. The disorder is highly heterogeneous and pediatric DCM often differs from adult DCM in etiologies, risk factors, and prognosis. Prognosis may be improving, likely secondary to specialized management. Both traditional and novel markers of risk have been studied in adults and are beginning to be applied to children with DCM. The major therapy for patients considered at risk remains implantable cardioverter-defibrillator (ICD) placement, although frequently this functions as a bridge to eventual transplantation.