A 59-year-old man presented with disc edema in the right eye. MRI demonstrated a lesion extending to the orbital apex. The lesion was biopsied and pathology revealed ectopic brain tissue. The presence of ectopic brain in the orbit is a rare finding. The few reports in the literature of sequestered ectopic brain in the orbit have all involved neonates or infants. We describe the first case of this rare tumor presenting in the orbit of an adult.