Calcific uremic arteriolopathy (CUA) is a rare but serious life-threatening complication of CRF that manifests as painful nonhealing eschars in association with panniculitis and dermal necrosis. This condition is being increasingly recognized and reported as a contributing factor to death in dialysis patients. The pathognomic lesion is vascular calcification with intimal arterial hypertrophy and superimposed small vessel thrombosis. Hyperparathyroidism and elevated concentrations of serum phosphate remain consistent clinical features of most cases reported. Controversy still exists regarding the role of parathyroidectomy in this condition with some studies suggesting improved outcome with surgical intervention. A number of potential new etiological factors have been identified including reduced serum levels of a calcification inhibitory protein alpha,2-Heremans-Schmid glycoprotein (Fetuin-A) and abnormalities in smooth muscle cell biology in uremia. Promising new treatment options including hyperbaric oxygen therapy and sodium thiosulfate infusion have been reported in case series. Benefits from biphosphonates and tissue plasminogen activator have also been reported. Overall these new treatment approaches and understanding of potential mechanisms underlying this important severe clinical condition offer new hope in the diagnosis and management of this severely morbid and often fatal condition.