Background: Retinal involvement in neurofibromatosis type-1 (NF-1) is rare.
Methods: We present a case report of a 49-year-old man with neurofibromatosis who developed bilateral macular lesions associated with gradual reduction of visual acuity.
Results: Electrophysiology revealed an absent pattern electroretinogram. Fluorescein angiography demonstrated mottled hyperfluorescence of the maculae in the early-phase fluorescein angiogram followed by late staining of the lesions.
Conclusions: Our patient had unusual bilateral macular lesions which had some features suggestive of adult-onset foveomacular vitelliform dystrophy. To the best of our knowledge, there have been no previous reports of such macular lesions occurring in association with NF-1.