An 18-year-old woman who presented with photosensitivity, butterfly rash and acute visual disturbance was diagnosed as SLE with retinopathy. The level of IFN-alpha in the cerebrospinal fluid (CSF) was markedly elevated. Her visual acuity recovered with high-dose prednisolone therapy. IFN-alpha in the CSF also reduced to within the normal range. The mechanism causing lupus retinopathy is not clearly understood. Although the association between lupus retinopathy and a high level of IFN-alpha has not been reported, the injection of IFN-alpha is known to frequently cause retinopathy in hepatitis patients. We discuss the possibility of IFN-alpha causing retinopathy in SLE patients.