Systemic and bone manifestations of CKD-MBD are highly prevalent and lead to significant consequences in children including growth failure and skeletal deformities, which contribute to long-standing disability. The exact pathophysiology of CKD-MBD remains to be identified and there are major gaps in the evidence for diagnosing the bone pathology and guiding treatment options. Measurement of PTH and biochemical bone markers is helpful but also lacks sensitivity and specificity, therefore therapeutic decisions should be based on the trend in levels rather than single measurements. Also, there is a significant shift from treating specific targets toward a more individualized approach to management. Continuing uncertainty of the radiological imaging of CKD-MBD in children further challenges therapeutic decisions. There is currently an absolute need for high-quality studies demonstrating the effectiveness of diagnostic and therapeutic interventions on patient-centered outcomes of children with CKD-MBD.