Calciphylaxis is a rare but devastating condition that has continued to challenge the medical community since its early descriptions in the scientific literature many decades ago. It is predominantly seen in chronic kidney failure patients treated with dialysis (uremic calciphylaxis) but is also described in patients with earlier stages of chronic kidney disease and with normal renal function. In this In Practice feature, we review the available medical literature regarding risk factors, diagnosis, and treatment of both uremic and non-uremic calciphylaxis. High quality evidence for the evaluation and management of calciphylaxis is lacking at this time due to its rare incidence, poorly understood pathogenesis, and the relative paucity of collaborative research efforts. We hereby provide a summary of recommendations developed by the Massachusetts General Hospital's Multi-disciplinary Calciphylaxis Team for calciphylaxis patients.