Gangliogliomas originating in the optic pathway are rare, with less than 20 cases reported in the literature. Diffuse, bilateral involvement of the entire optico-chiasmatic pathway is exceptional. We report a case of suprasellar ganglioglioma that involved bilaterally the entire pregeniculate optic pathway. The patient presented with visual deficit, nystagmus, papilledema and acute biventricular hydrocephalus secondary to intraventricular cyst that required urgent surgery. Endoscopic fenestration of the tumoral cyst allowed control of hydrocephalus and decompression of the visual pathway. Through microsurgical procedure by pterional approach, partial removal of the tumor and histological diagnosis were accomplished 1 week later. The patient was managed with chemotherapy and radiation therapy. He presents stable residual disease at 4-year follow-up. Embryological origins, histological features, neuroradiological appearance, management and prognosis of optic pathway gangliogliomas are reviewed.
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