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A rare case of histologically verified orbital pseudotumor with intracranial extension in a 33-year-old male is reported. Preoperative radiographs showed the mass extending beyond the orbit and involving the bilateral cavernous sinuses. The intracranial lesion at biopsy surrounded the right internal carotid artery above the right cavernous sinus. Cerebral angiography showed complete occlusion of the cervical portion of the right internal carotid artery. Possibly, multiple lesions had extended contiguously through the venous system around the cavernous sinus, with subsequent involvement of the internal carotid artery. This case suggests that orbital pseudotumor may not be a separate clinical entity, but a part of the syndrome caused by inflammation of the venous system around the cavernous sinus.