A neonate presented with a dilated pupil of the left eye. Magnetic Resonance Imaging (MRI) showed a mass in the left inferior part of the orbit. Incisional biopsy was performed. Histopathological examination revealed ectopic brain tissue. The patient had a 5-year follow-up without any growth on MRI. Ectopic brain tissue in the orbit is a rare finding in the literature, almost all involved infants or neonates. Pathogenesis is still unknown.