Abstract
A 9-month-old girl who presented with bilateral pseudodendritic keratitis was unresponsive to antiviral therapy. A diagnosis of tyrosinemia type II (Richner-Hanhart syndrome) was confirmed by high plasma tyrosine levels (2017 mol/L). After the patient was placed on a low-protein diet that was supplemented with a tyrosine-restricted and phenylalanine-restricted formula (Mead-Johnson 3200 AB), the ocular symptoms and signs resolved. Tyrosinemia type II should be suspected in cases of resistant forms of bilateral dendritic keratitis. Timely initiation of dietary regimen will result in the rapid resolution of signs and symptoms, and prevent serious complications.