A case of a 53-year-old chronically uraemic female patient who suddenly developed widespread cutaneous necrosis on both thighs is presented. On the basis of the typical histopathological features a diagnosis of calciphylaxis was made and supported by elevated serum parathyroid hormone levels. Calciphylaxis is a rare and serious disease, mostly seen in uraemic patients subjected to chronic haemodialysis. Typical features are calcifying panniculitis and thrombosis of small to medium-sized blood vessels followed by necrosis of the skin. The poor prognosis is due to systemic calcifications of visceral organs. When acute cutaneous necrosis of the skin is encountered in patients with chronic uraemia calciphylaxis should always be considered as a possible cause in the differential diagnosis. Determination of the serum parathyroid hormone level is recommended.