Heterotopic brain tissues are a group of extracerebral neuroglial tissues. Heterotopic brain tissue in the orbit remains a rare clinical entity. This article presents a 7-year-old male child who presented with severe globe displacement, proptosis, and vision loss in the right eye. The orbital imaging showed a huge orbital cystic mass displacing the globe. The cyst was excised entirely from the orbit. The histopathological investigations revealed the presence of a cystic lesion containing brain tissue that was immune reactive for S-100 and glial fibrillary acidic proteins. The diagnosis was confirmed to be heterotopic brain tissue due to the lack of visible bony defect. The relevant literature was also reviewed.
Keywords: Cyst; heterotopic brain tissue; orbital tumor.