After a brief survey of the forms of cranial meningoencephaloceles, the current opinions on their development, symptoms and surgical therapy, a report is given on the case of a five-year-old boy suffering from a sphenoethmoidal meningocele of the size 3 x 3 x 4 cm. There was at the same time a wide median palate cleft, obviously a consequence of the meningoencephalocele. A further finding presented by the boy was a neuroradiologically diagnosed agenesis of the Corpus callosum and a median cheiloschisis. This suggests a central development disturbance as a primary cause. The attempt to perform an intracranial occlusion of the defect of the base, which was repeated twice, was not successful. It was only by a transpalatinal-transnasal extradural intervention (reposition of the meningocele after removing the epipharyngeal mucosa, blocking of the bony defect with a perforated tantalum plate) that the removal of the meningocele was successful. The closure of the palate cleft was then made by the oral surgeon. This surgical intervention may also be considered for the treatment of the transsphenoidal meningoencephalocele, which also presents many problems.