Introduction: Optic pathways cavernomas are rare vascular hamartomas that can present either with an acute chiasmal syndrome or slowly progressive visual loss.
Observation: A 29-year-old patient presented with mild unilateral visual loss of rapid onset and monocular left temporal hemianopia. MRI disclosed a heterogenous enhancing optochiasmal lesion. Work-up found no evidence for an inflammatory, infectious or tumoral disease and therefore a neurosurgical approach of the lesion allowed diagnosis of cavernoma, confirmed by pathological examination. Successful resection resulted in partial recovery of the visual field.
Conclusion: Optochiasmal cavernomas are rare, removal can improve visual outcome.