• Xiang Yang, Jiagang Liu, Yanming Ren, Seidu A Richard, and Yuekang Zhang.
• Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan, China.
• Medicine (Baltimore). 2017 Nov 1; 96 (47): e8754.

RationaleRosai -Dorfman disease (RDD) is a rare, idiopathic, and non-neoplastic histioproliferative disease with distinctive entity of unknown etiology. Central nervous system (CNS) RDD is uncommon, hence, isolated intracranial RDD is extremely rare. So far only 6 cases of CNS RDD with the lesions originating from petroclival region have been reported. We present a case of isolated intracranial RDD mimicking petroclival meningioma.Patient ConcernsA 14-year-old girl was admitted at our hospital with a 3-month history of dizziness, slowly progressing headache, and 2-month history of instability in walking. Cranial nerve deficits, including left facial paralysis, left facial numbness and left hearing loss, were evident on examination.DiagnosesInitial diagnosis of petroclival meningioma was made according to preoperative magnetic resonance imaging.InterventionsThe lesion was resected subtotally and pathology confirmed RDD. The patient received gamma-knife treatment for the residual lesion.OutcomesThe patient recovered well and the residual lesion significantly retrogressed on follow-up images.LessonsPreoperative diagnosis of petroclival RDD is full of challenges. Although surgical resection of lesions is an effective treatment option, total resection is not highly recommended because the surgery-related defect must be minimal. Patient with residual lesion can be put on steroid therapy and/or radiotherapy, especially for IgG4 positive subset of RDD.Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

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