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A consequence of chronic and end-stage kidney disease is a higher risk of calcium deposition in sites other than the bones. The authors of this review outline current understanding of the pathogenesis, presentation, diagnosis, and treatment of this group of disorders. KEY POINTSExtraosseous calcifi cation is a broad term that encompasses vascular calcifi cation, soft tissue calcifi cation, and calciphylaxis, all of which are seen in patients with end-stage kidney disease.The pathogenesis of extraosseous calcifi cation is an active process involving a complex interplay of abnormal electrolyte levels, cell differentiation, and dysregulation of many biochemical pathways.Vascular calcifi cation is predominantly diagnosed incidentally, while soft tissue calcifi cation and calciphylaxis are diagnosed on the basis of radiographic and clinical presentation, sometimes requiring biopsy.Management is based on low-quality evidence and includes maintaining a neutral calcium balance, correcting hyperphosphatemia, and controlling comorbidities. Surgical and other nonmedical therapies may help somewhat in managing calciphylaxis and soft tissue manifestations.

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