Uremic leontiasis ossea is a rare condition, reported in patients with severe renal disease. Patients present with progressive enlargement of facial bones - in particular, the maxillary and mandibular bones. Rarity of this condition leaves clinicians puzzled on initial evaluation and management. Herein, we present a 31-year-old man diagnosed with uremic leontiasis ossea. The report aims to review the pathophysiology of the condition as described in the literature, the patient presentation and imaging modalities used to investigate, and classical findings seen in patients with uremic leontiasis ossea. Finally, we briefly touch base on the reported regimens used to prevent and manage this condition.