Transspheinoidal meningoencephalocele is a rare presentation of dysraphism of the neural tube. It is characterized by the herniation of the neural tissue through a bony defect in the sphenoid bone. The clinical presentation is variable. It may be assymptomatic or it may include an upper airway obstruction, rhinorrhea, meningitis, hypothalamic dysfunction and optic anomalies. The surgical treatment is controversial. We describe the case of a 7-year-old boy who presented a pulsate structure filling the palate, palate digenesis and hypertelorism. The diagnosis of transsphenoidal transpalatal meningoencephalocele was confirmed by a computerized tomography and a magnetic resonance imaging. The child was operated on by the transpalatal/transspheinoidal approach with a good result.