Resumen:
The optic nerve glioma is a very uncommon entity in adult patients, with little information about its natural history and
therapeutical management. We report the case of a 27-year-old patient with acute involvement of the left optic nerve due
to intratumoral hemorrhage, a very uncommon form of presentation in this type of tumor. Resection was performed using
an extended transsphenoidal endoscopic approach, with functional preservation of the contralateral optic pathway. The
histopathology confirmed positive pilocytic astrocytoma with KIAA 1549-BRAF rearrangement and without BRAFV600E
mutation. Considering the histopathology and molecular biology, the contralateral visual stability and the wide surgical
resection, it was decided not to perform further treatment. The purpose of this decision was to avoid additional damage to the
chiasm, contralateral optic nerve and/or hypothalamus. Given the limited data available in medical literature, the report of this
case could contribute with additional information on the management and therapeutic approach of this type of tumors.