The authors present a single case of atypical presentation of Vogt-Koyanagi-Harada disease. A 42-year-old female presented with unilateral visual loss associated with systemic symptoms of fever and headache. Initial ophthalmic examination revealed a unilateral neuroretinitis, however, investigations for infectious and non-infectious causes of neuroretinitis were negative and unremarkable. On further examination, retinal imaging suggested bilateral involvement and lumbar puncture results demonstrated pleocytosis, consistent with a diagnosis of Vogt-Koyanagi-Harada disease. The patient was treated with intravenous steroids which resulted in a prompt resolution of her symptoms. To the authors knowledge this is the first reported case of unilateral neuroretinitis caused by this disease and the first reported case of this mode of presentation. The authors conclude that Vogt-Koyanagi-Harada disease should be considered in the differential diagnosis of neuroretinitis.