A patient is described with an orbital varix arising from the right superior ophthalmic vein, associated with ophthalmoplegia and severe pain, and without proptosis. The varix was detected using computerized tomography and orbital phlebography, and at surgery was verified as a venous aneurysm. During the operation, a pearly phlebolith was found. Histological examination of the varix revealed multiple ectatic venous channels. The etiology of this unusual clinical manifestation and the treatment of the patient are briefly discussed.