Calciphylaxis is a rare but highly morbid disorder of vascular calcification and skin necrosis, affecting 1% to 4% of adults with end-stage renal disease. Only three affected children have previously been described. We report an unusual instance of fatal calciphylaxis involving a 10-year-old boy, in the setting of sarcoidosis-induced end-stage renal disease. A review of pediatric calciphylaxis cases suggests: (a) increased risk in boys with end-stage renal disease and secondary hyperparathyroidism; (b) frequent distal extremity and visceral organ involvement, (c) worse prognosis with acral necrosis, and (d) possible increased resistance to medical treatment compared to adult patients. Hyperesthetic pain is a common sentinel symptom, and soft tissue x-rays or xeroradiography or both may aid in the diagnosis of pediatric calciphylaxis. Parathyroidectomy in combination with supportive medical management may be the best treatment option in stopping the progression of disease in the pediatric population.