In young adults with onset of chronic kidney disease in childhood, cardiovascular disease is the most common cause of death. The likely reason for increased cardiovascular disease in these patients is a high prevalence of traditional and uremia-related cardiovascular disease risk factors during childhood chronic kidney disease. Early markers of cardiomyopathy, such as left ventricular hypertrophy and left ventricular dysfunction, and early markers of atherosclerosis, such as increased carotid artery intima-media thickness, carotid arterial wall stiffness, and coronary artery calcification, frequently are found in this patient population. The purpose of this review is to provide an update of recent advances in the understanding and management of cardiovascular disease risks in this population.