Primitive orbital meningocele is a rare congenital malformation. It is defined as a herniation of meninges into the orbit through a congenital defect in the orbital bones. In meningoencephalocele, there is atrophic brain tissue in the herniated meningeal sac. We report the cases of two babies who presented with a medial orbital tumoral syndrome. Computed tomography showed a cystic tumor close to a bony defect in the anterior frontoethmoidal junction. A transcranial approach confirmed the diagnosis of meningoencephalocele; it was resected and the bony and dural defects were closed. The outcome was uneventful. Orbital meningoencephalocele is probably linked to an abnormal closure of the rostral neuropore. It generally presents in the first months of life with very suggestive radiological and clinical features. Surgical treatment using a transcranial approach is indicated in all cases and provides definitive recovery.