As in older adults, cardiovascular disease is the most important cause of death in adolescents and young adult patients with end-stage renal disease (ESRD) since childhood. This concerns patients on dialysis as well as transplant patients, despite the fact that a long duration of dialysis during childhood is an extra mortality risk factor. Left ventricular hypertrophy (LVH), aortic valve calcification, and increased arterial stiffness, but not increased arterial intima media thickening, are the most frequently observed alterations in young adult survivors with childhood ESRD. In transplanted patients a concentric LVH as a result of chronic hypertension is mostly observed; in dialysis patients a more asymmetric septal LVH is found as a result of chronic volume overload. These results suggest that in children and young adults with ESRD chronic pressure and volume overload, a high calcium-phosphate product, and chronic inflammation, but not dyslipidemia, play a role in the development of cardiovascular disease.