On 7 July 1993, a 61 year-old female presented a seven-day history of double vision and retro-ocular pain. Neurological examination on admission demonstrated right oculomotor palsy and pain in the right frontal nerve. Routine CT scan and MRI disclosed no abnormalities. Her symptoms deteriorated progressively and increased in severity, resulting in complete ptosis and visual disturbance in the right eye, for which she was hospitalized. Angiography also failed to demonstrate any lesion. Herrical CT scan demonstrated a small mass lesion at the right orbital apex. A frontotemporal extradural approach operation was performed on July 6, 1993. The anterior clinoid process and minor wing of the sphenoid were drilled to expose the optic canal and the superior orbital fissure. When the periorbita just beside the optic sheath was opened, a large vein was observed to be impinging on the optic nerve and nerves which pass through the superior orbital fissure. This vein was carefully dissected, coagulated and removed. The postoperative course was uneventful. Her severe retro-ocular pain completely disappeared immediately after the operation. Although complete remission of ptosis and return of eye movements to normal were noted, her visual acuity is at present limited to hand movement. Orbital varix is a common cause of unilateral intermittent proptosis. However, there has been no report in the literature of orbital varix with orbital apex syndrome as the initial clinical appearance. In this type of patient with orbital varix and retrobulbar hemorrhage or impairment of intraorbital nerves, surgical intervention using a transcranial approach to the lesion should be undertaken.