Purpose of review Cardiovascular disease (CVD) begins early in the course of chronic kidney disease (CKD) and is the most common cause of death even in children with CKD. Mechanisms of CVD development are poorly understood. This review focuses on the role of dysregulated homeostasis of calcium (Ca), phosphorus (P), parathyroid hormone (PTH) and vitamin D in ectopic vascular calcification. Recent findings Converging evidence from clinical, epidemiological and translational research studies suggest that uraemic vasculopathy and ectopic vascular calcification begin in predialysis CKD, progress inexorably when the child is on dialysis and may only partially reverse after successful transplantation. Although several 'traditional' and CKD-related risk factors are involved, dysregulated mineral metabolism plays a key role in the development and progression of childhood CVD. Summary Children with CKD carry a high burden of cardiovascular risk factors from early CKD stages. Thus, identification of key modifiable risk factors and implementation of appropriate preventive measures must begin early in CKD. As vascular calcification is a highly regulated cell-mediated process with several promoters and inhibitors of calcification, it may be possible to inhibit progression or even reverse established vascular calcification.