Schwannoma (neurilemoma) arising from the ciliary body and choroid is a rare form of intraocular tumor and usually misdiagnosed clinically as amelanotic melanoma. This case report describes a 73-year-old woman who developed a large nonpigmented intraocular tumor of the choroid in the left eye and underwent enucleation with a clinical diagnosis of malignant melanoma. The tumor consisted of spindle-shaped cells with an indefinite cytoplasm and twisted nuclei, which had positive immunoreactivity for S-100 protein, vimentin, glial fibrillary acidic protein and neural cell adhesion molecule. A continuous basal lamina encompassing the tumor cells was demonstrated by immunostaining for laminin and type IV collagen. Ultrastructurally, tumor cells with scant organelles had delicate cytoplasmic processes apposing each other, all indicative of Schwann cell origin. In reexamination of fluorescein angiograms, ultrasonograms and magnetic resonance images, no findings were incompatible with a diagnosis of choroidal melanoma. Histopathological studies showed that the tumor was a schwannoma. At present, we do not have a benchmark for the clinical diagnosis of intraocular schwannoma.