A 25-year-old man presented with an intraorbital encephalocele manifesting as progressive left pulsatile exophthalmos. He had a history of frontal lobe contusion from a motorbike accident 10 years before the onset of the symptom. Computed tomography and magnetic resonance imaging revealed an oval-shaped defect in the left orbital roof with an underlying intracranial cystic lesion, herniated into the orbit. Intraoperative findings included disruption of the dura mater around the bony defect. The loculated arachnoid membrane and protruding brain tissue were excised with primary dural closure and reconstructive cranioplasty with a titanium mesh. The postoperative course was uneventful, and the pulsatile proptosis disappeared immediately after the procedure. Intracranial cyst may be important in the development of progressive pulsatile exophthalmos and intraorbital encephalocele.