Cavernous angioma of the optic chiasm or optic nerve is extremely rare. We report the case of a 58-year-old woman with cavernous angioma of the optic chiasm. The lesion was totally removed through the eyebrow keyhole approach, which allowed appropriate intraoperative exploration of the optic chiasm and related structures. The present case confirms that a cavernous angioma located in the optic chiasm can be totally resected without further impairment of visual function.