Summary

Schwannomas located in the eye are extremely rare and mainly arise from ciliary nerves. Ocular schwannoma usually present in the choroid, ciliary body or rarely sclera as a benign solitary amelanotic lesions. A 41-year-old woman presented with a history of decreased vision in her right eye of 3 months duration. She had an amelanotic lesion in the nasal side of left fundus. After all work-ups, malignant melanoma was suspected and enucleation was done. In clinicopathologic examination, findings were in favour of intrascleral schwannoma. Despite new modern diagnostic modalities, there are still some fundus lesions that are easily mistaken for amelanotic melanoma. Diagnostic procedures such as fine needle aspiration should be considered for equivocal lesions in the fundus that are not definitely diagnosed before surgery.