Necrotizing livedo reticularis is an infrequent, life-threatening complication of chronic renal failure. Since Selye's studies in 1962, calciphylaxis, i.e. acute calcium deposition in tissue, is considered the main pathomechanism, especially because hyperparathyroidism are very frequently present. However, other etiological and/or triggering factors, such as coagulation disorders, direct cellular toxicity of parathormone or calcium on endothelium, might be involved, acting perhaps in a cumulative way. We report a case with a circulating anticoagulant which supports this hypothesis.