Calciphylaxis is a rare, potentially life-threatening syndrome characterized by progressive and painful skin ulcerations associated with medial calcification of medium-sized and small cutaneous arterial vessels. Calciphylaxis primarily affects patients on dialysis or after renal transplantation; however, exceptions have been reported in patients with normal renal function and in association with chronic-inflammatory disease, malignancy or primary hyperparathyroidism. Clinically, calciphylaxis is associated with a high mortality of up to 80%. Superinfection of necrotic skin lesions with subsequent sepsis significantly contributes to this outcome. Many patients also suffer from cardiovascular disease characterized by calcification of larger arterial vessels.