Perineuriomas are infrequent tumors that may be intraneural or extraneural in soft tissue with no apparent relation with a nerve. Some cases of soft tissue perineurioma may have a retiform pattern, but this pattern has not been described to date in intraneural perineurioma. We report a case of a unique perineurioma arising in a nerve of the neck that had a distinctive reticular pattern and was intraneural. The patient was a 21-year-old woman who had a lump on the left side of the neck abutting the vena jugularis. The tumor had features of both reticular perineurioma and intraneural perineurioma with pseudo-onion bulb pattern. The tumor cells were positive for epithelial membrane antigen. The patient is free of tumor 6 months after operation.
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