Leontiasis ossea is a rare medical condition which is characterized by an overgrowth of the facial and cranial bones secondary to chronic renal failure and secondary hyperparathyroidism. We reported a case of leontiasis ossea with history of secondary hyperparathyroidism due to end-stage renal disease on regular hemodialysis. A 37-year-old female with end-stage renal disease from lupus disease on regular hemodialysis complaining of progressive facial deformity for two years causing dyspnea, dental malocclusion and dysarthria with the final diagnosis of uremic leontiasis ossea. It is important to recognize features of leontiasis ossea, as it may result in life-threatening upper airway obstruction and compressive cranial neuropathy while after parathyroidectomy, facial changes can be stabilized or improved mildly.