The craniofacial skeleton represents a peculiar target of hyperparathyroidism in patients with end-stage renal disease who exhibit a dramatic pattern of uremic leontiasis ossea. Scant information regarding this condition is available in the renal literature, as the extreme and typical manifestations of leontiasis ossea have been described in only a small series of patients. We herein report a case of significant amelioration of massive modification of the facial appearance of a 30-year-old uremic Chinese woman with severe skeletal deformities who underwent total parathyroidectomy with a forearm autograft concurrently with effective drug treatment. This report may shed light on how to better understand and treat this metabolic derangement.