A 20-month-old infant found to have a blind eye with neovascular glaucoma was thought to have a neoplasm of the optic nerve for which enucleation was performed with resection of a long segment of optic nerve. Microscopic examination revealed the enlarged optic nerve and optic papilla to be infiltrated densely by histiocytic cells, including Touton giant cells, all containing large amounts of neutral fat. The histologic picture was indistinguishable from that of juvenile xanthogranuloma. Occlusion of the central retinal vessels had led to hemorrhagic infarction of the retina and neovascular glaucoma. Thorough clinical and laboratory investigations repeated over a period of more than 2 1/2 years failed to disclose any evidence of a systemic disease, and the child has remained in good health. This is believed to be a unique case of juvenile xanthogranuloma of the optic nerve and disc.