Background: Hemangioblastomas are rare benign tumours that are most commonly detected in the subtentorium or spinal cord. Optic nerve hemangioblastoma is very rare and is most commonly associated with Von Hippel-Lindau (VHL) syndrome.
Case presentation: Here, we report a case of hemangioblastoma of the optic nerve with bilateral frontal lobe oedema without VHL syndrome, which has not yet been reported. A 51-year-old woman presented with progressive and painless deteriorating vision in the left eye. Magnetic resonance imaging showed a mass at the back of the left orbital optic nerve. Endoscopic-assisted intraorbital tumour resection was performed successfully. The pathological diagnosis was left optic nerve hemangioblastoma.
Conclusions: This is the first reported case of optic nerve hemangioblastoma (HBL) with bilateral frontal lobe oedema.
Keywords: Hemangioblastoma; Optic nerve; VHL syndrome.