Primary melanocytic tumors of the central nervous system (CNS) are rare neoplasms that range from benign melanocytomas to aggressive malignant melanomas. Although meningeal melanocytomas are generally considered indolent lesions, malignant transformation and distant metastasis can occur.

We report the case of a 33-year-old male with a bulbopontine meningeal melanocytoma who developed systemic metastases, culminating in a fatal outcome. Despite initial histopathologic features of benignity and absence of BRAF mutation, the lesion showed aggressive behavior.

This case underscores the diagnostic pitfalls associated with primary CNS melanocytic tumors and highlights the importance of long-term vigilance, even for histologically benign lesions.

This study was conducted in accordance with institutional ethical standards and the Declaration of Helsinki. Ethical approval was obtained from the local ethics committee of our institution following a formal review of the research protocol.

Written informed consent was obtained from the patient's next of kin for publication of this case report and any accompanying images.

M.A.I. is currently affiliated with Department of Pathology, Acibadem Mehmet Ali Aydinlar University Faculty of Medicine, Istanbul, Türkiye.

Received: 14 June 2025

Accepted: 21 September 2025

Article published online:
03 December 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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