Choroidal schwannoma in a 6-month-old girl

Choroidal schwannomas are exceedingly rare in children, with only 6 cases reported in children younger than 18 years of age and none in those younger than 9 years. We report a 6-month-old infant presenting with a large noncalcified amelanotic mass with secondary glaucoma that mimicked an atypical retinoblastoma, leading to emergent enucleation for therapeutic and diagnostic purposes. Pathology revealed a total retinal detachment, glaucomatous damage, and a large mass arising from the peripapillay posterior choroid with areas of Antoni A pattern and S-100 staining consistent with the diagnosis of an intraocular schwannoma. This is the first intraocular schwannoma described in an infant.