A patient is presented who had unrecognised Wilson's disease. He had developed a clinically obvious Kayser-Fleischer ring in only one eye. The eye without the corneal ring had been injured in childhood and had a low intraocular pressure. Possible mechanisms for formation of a Kayser-Fleischer ring are reviewed and the lack of Kayser-Fleischer ring in this case is discussed.

Wilson's Disease (WD) is a rare, autosomal, recessive, inborn error of the copper metabolism, which is caused by a mutation in the copper-transporting gene, ATP7B. The presentation is usually neurologic or hepatic, which is seen in 40% of the patients. The diagnosis depends primarily on the clinical features, the biochemical parameters and the presence of the Kayser - Fleischer ring. Here, we a...

Although the brownish area at the corneal periphery in the region ofDescemet's membrane was first described by Kayser (I902), and Fleischer (I9I2) recognized it as a part of Wilson's disease (hepato-lenticular degeneration), there have been no previous reports in the ophthalmic literature of the United Kingdom of a pathological study of the lesion which is now recognized as a pathognomonic find...

Here we describe a case of a 22-year-old woman who presented with acute liver failure and Kayser-Fleischer rings suggesting the diagnosis of Wilson's disease.