Peripheral nerve sheath tumors of the uveal tract arise mainly in the sheath of peripheral nerves that are found in the uvea. The ciliary nerve is most often affected, and hence, these tumors tend to be located anteriorly in the ciliary body or peripheral choroid in the anatomic location of the long ciliary nerve. There are several systemic predisposing risk factors, which include schwannomatosis, neurofibromatosis 1 (NF1) and 2, and other rare genetic syndromes (PTEN syndrome). The benign tumors can be subclassified as ganglioneuroma, neurofibroma, and schwannoma.