Snapshot A 44-year-old woman presents to her ophthalmologist for a follow-up visit. She was recently diagnosed with Wilson disease when she initially presented to the hospital for acute liver failure. She currently does not report any acute complaints and says she has been taking her D-penicillamine with zinc salts as prescribed. She undergoes a slit-lamp examination, which demonstrates gray-green deposition in Descemet membrane in the cornea. Introduction Overview an ocular manifestation of Wilson disease, an autosomal recessive disorder of impaired copper excretion can be seen in other clinical conditions primary biliary cirrhosis neonatal cholestasis Epidemiology Risk factors family history etiology Pathophysiology copper deposition in Descemet membrane in the cornea Genetics inheritance pattern autosomal recessive mutations chromosome 13 gene ATP7B gene protein copper-transporting P1-type ATPase Studies Patients should be evaluated for Wilson disease Differential Biliary obstruction differentiating factors obstruction found on abdominal imaging (e.g., abdominal ultrasound) DIAGNOSIS Clinical diagnosis Treatment Medical D-penicillamine or trientine indication first-line in symptomatic patients decreases disease progression of Wilson disease chelating agent zinc salt indication helps with symptom improvement impairs copper absorption Complications Complications of Wilson disease, which include acute liver failure (most common) infertility chondrocalcionosis Prognosis Fatal, if not treated