A congenital intranasal encephalocele is repaired at 23 months of age via a transnasal endoscopic approach. To the best of our knowledge, this is the youngest patient reported in the literature to be treated successfully with this approach. The technique and methods are described. This case highlights the importance of endoscopic approaches to encephaloceles resulting in less morbidity than a traditional craniotomy. Continued advances in technology and skill will increase the feasibility of transnasal endoscopic skull base surgery in younger patients. © 2005 Published by Elsevier Ireland Ltd.