Uremic calciphylaxis is a rare disease that affects patients with chronic end-stage renal disease. It is a pathology of the microvessels of the dermis and hypodermis which are calcified and whose thrombosis leads to skin necrosis. Calciphylaxis lesions can be distal and axial. They lead to pain, infection and are associated with denutrition and in high mortality rate (40-80% at 1 year). This general review describes the clinical and para-clinical presentations of calciphylaxis. It summarizes the current knowledge on its pathogenesis and the therapeutical options that can be proposed to improve the management and attempt to reduce the mortality of patients with uremic calciphylaxis.