Cardiovascular disease (CVD) is a major cause of death in pediatric patients with chronic kidney disease (CKD). Both types of traditional and CKD-related risk factors for CVD are prevalent in children with CKD. Many of these factors are associated with endothelial dysfunction (ED) and nitric oxide (NO) deficiency. ED with NO deficiency is both a cause and consequence of CKD. Hence, ED links CVD to pediatric CKD because ED plays a major role in the development of CVD. This review discusses how the risk factors for CVD contribute to ED and NO deficiency, leading to CVD in pediatric CKD. With early detection of ED by new non-invasive methods and restoration of NO bioavailability through different therapeutic approaches, the morbidity and mortality of CVD in pediatric CKD patients can be reduced.