A 15-year-old female patient with progressive pulsatile exophthalmos caused by intraorbital encephalocele was evaluated with computed tomography (CT) and magnetic resonance imaging (MRI) in our clinic. She had no history of trauma or reconstructive surgery. When she was a little girl, she had undergone surgery for congenital glaucoma on the right eye. On the three-dimensional image of CT, a hypoplasic bone defect was observed in the greater wing of the right sphenoid bone. MRI and CT scan showed herniation through this defect of the arachnoid membrane and protruded cerebral tissue into the right orbita. Intraorbital encephalocele is an important entity that can cause pulsatile exophthalmos and blindness.