Background Hemangioblastomas are benign, highly vascular World Health Organization (WHO) grade 1 tumors that most commonly arise in the cerebellum. Brainstem involvement is uncommon, and lesions originating from the cervicomedullary junction with extensive caudal cystic extension into the cervical spinal cord are exceptionally rare, posing significant diagnostic and microsurgical challenges.

Case Description A 39-year-old man presented with a 2-year history of progressive paresthesia involving both upper limbs, right-sided weakness, gait imbalance, and cerebellar symptoms. Magnetic resonance imaging demonstrated a vividly enhancing mural nodule arising from the dorsal cervicomedullary junction associated with an elongated intramedullary cystic cavity extending caudally to the C3 vertebral level, accompanied by cord edema and syringomyelia. Evaluation for von Hippel–Lindau disease was negative. The patient underwent gross total microsurgical excision through a midline suboccipital craniectomy with C1 laminectomy. Histopathological examination confirmed hemangioblastoma (WHO grade 1). The postoperative course was uneventful, with significant neurological improvement during follow-up.

Conclusion Primary cervicomedullary hemangioblastoma with extensive cervical cystic extension is an exceptionally rare lesion. Careful preoperative imaging evaluation, meticulous microsurgical technique, and complete tumor excision can achieve favorable neurological outcomes. Comparison with previously reported cases highlights the rarity of this presentation and supports gross total resection as the treatment of choice whenever safely feasible.

Received: 18 February 2026

Accepted after revision: 20 July 2026

Accepted Manuscript online:
24 July 2026

Article published online:
03 August 2026

© 2026. 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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