A cephalocele is defined as a herniation of cranial contents through a defect in the skull. Cephaloceles are classified according to their contents and location. We have reviewed a total of 112 patients with cephaloceles, 51 of whom had sincipital meningoencephaloceles (fronto-ethmoidal meningoencephaloceles). This group is distinctive in its demographic distribution, in the effect on growth of other facial structures, and in the combined craniofacial approach needed to treat them. This review is based on the sincipital encephaloceles with the other cephaloceles included for completeness. Despite many theories, the cause of congenital cephalocele is not known. Preoperative work-up includes 3-dimensional computed tomography scan of the facial skeleton, and surgical management is multidisciplinary in nature. The aim is to remove the lesion before the deformity has time to greatly distort facial growth, which appears to realign itself after surgery. The 50 patients who underwent surgery for fronto-ethmoidal encephalocele all survived with minimal complications.