Transsphenoidal cephalocele is a rare lesion traditionally managed by either an intracranial approach or by a combined intracranial and extracranial approach. A two-year-old boy presented with a transsphenoidal cephalocele manifesting as apneic-cyanotic spells accompanied by breathing difficulties due to nasal obstruction after birth. Neuroimaging revealed a cystic mass extending from the pituitary fossa into the nasopharynx through the sphenoid sinus. Nasal endoscopy confirmed the diagnosis of transsphenoidal meningocephalocele. The lesion was managed with an endoscopic endonasal approach. The cephalocele and its contents were passively reduced by applying bipolar cautery to the overlying mucosa. Once reduced, the mucosa was incised and removed from the underlying dura to allow further reduction of the dural sac and its contents. The dural sac was amputated partially using microscissors, followed by repair of the dural defect. The patient developed mild diabetes insipidus after surgery. Follow-up evaluation demonstrated resolution of the preoperative symptoms without lasting morbidity. This procedure is safe, less time consuming than the transcranial approach, and efficacious. (C) 2010 Elsevier Ltd. All rights reserved.