A spectrum of retinal features has been recognised in association with incontinentia pigmenti. The majority of reported cases describe a rapidly progressive proliferative retinopathy, often leading to retinal detachment, emphasizing the potentially blinding nature of the disease. In some instances, the retinopathy has been noted to be less severe but follow-up has been short. We describe a young girl with incontinentia pigmenti in whom the retinopathy has shown a fluctuating but stable course over 13 years without treatment.