A 42-year-old man with neurofibromatosis type 1 underwent a giant facial neurofibroma resection and enucleation of the right eye. He was found to have a diffusely thickened and cellular choroid containing individual or small clusters of large ganglion and medium-sized neuronal cells. The nomenclature of this lesion is difficult; however, the term choroidal ganglioneuronal hamartoma has been favored due to the lack of proliferative activity and diffuse nature of the lesion arguing against a neoplastic etiology. This case highlights an interesting presentation of neurofibromatosis type I.
