Perineuriomas are benign, rare neoplasms of the nerve sheath, basically in two forms: intraneural and extraneural. Extraneural forms are mainly found in the trunk and limbs, while visceral organs are rarely affected. To date, there have been no previous reports in the literature of bladder perineurioma. In this case, we report a young adult with hematuria and bladder tumor which after surgical resection and immunohistochemical study was confirmed to be a perineurioma. Therefore, this should be included in the differential diagnosis during the analysis of resections of bladder tumors.