Calciphylaxis is a complication of chronic kidney disease characterized by necrotic lesion in the skin. Histological examination reveals microcalcification of medium-sized blood vessels. We report on a 21-month-old girl with end-stage renal disease with severe calcium-phosphate imbalance. Calciphylaxis process started when she received calcium gluconate intravenously the day before the surgery to correct hypocalcemia and continued progressively despite peritoneal dialysis and forced stopping calcium-containing medication. Pamidronate, 0.5 mg/kg/d, was administered for 6 days and then once a week for 5 weeks. After 1 week, the skin lesion started to heal and circulation improved, and after 6 weeks, all skin lesions completely recovered. Pamidronate was effective to stop calciphylaxis in this case with advanced renal insufficiency and severe calcium-phosphate imbalance. Medical or surgical debridement are not suggested and lesions might recover without scar by pamidronate.