Neurothekeoma is a benign cutaneous nerve sheath tumor usually located in the head and neck region. To date, only a few cases of intracranial location have been reported in the literature. We report an extremely rare case of intracranial neurothekeoma located in the brain parenchyma of the middle cranial fossa. A 55-year-old female patient was operated on at our clinic for a right temporal cystic mass lesion with het erogenic contrast enhancement. Histologically, the tumor had a multinodular hypercellular appearance with a myxoid matrix, peripheral fibrosis, and sclerosis. It was characterized by spindle and epithelioid mononuclear cells, immunochemically positive with diffuse S-100, vimentin, GFAP, PGP 9.5, and NSE, as characteristics of cellular neurothekeomas. After a total surgical resection, there was no need for further adjuvant chemotherapy or radiotherapy. Because there were still unclear aspects regarding the origin, pathogenesis and management of the intracranial neurothekeomas, we decided to contribute to the literature with this case report. (C) 2016 Elsevier Masson SAS. All rights reserved.