Tumors that originate from the nerve sheath comprise diverse groups -perineuroma, neurofibroma and schwannoma. The epithelioid variant of these tumors is uncommon and mostly seen in malignant counterparts. Although this type of morphology is well recognized in peripheral nerve sheath tumors, its presence in the central nerves is rarely reported. Very few cases of nerve sheath tumors have been reported with collagen-rich stroma. Here we report an extremely rare case of nerve sheath tumor with epithelioid morphology and collagen rich stroma. To the best of our knowledge this finding in the spinal cord has not been reported thus far.