Background: Congenital skull base defects have traditionally been treated via an intracranial approach. Recent advances in endoscopic management have made minimally invasive extracranial approaches feasible, with less morbidity. Objective: To determine the success of endoscopic treatment of congenital cerebrospinal fluid leaks and encephaloceles. Main Outcome Measures: Retrospective review of congenital cerebrospinal fluid leaks and encephaloceles treated from January 1, 1992, to December 31, 2003. Data collected include demographic characteristics, presenting signs/symptoms, site of the skull base defect, surgical approach, repair technique, and clinical follow-up. Results: Eight patients were treated via the endoscopic approach for congenital cerebrospinal fluid leaks and encephaloceles. The average age at presentation was 6 years (range, birth to 18 years). Three patients presented with meningitis (average age, 6 years), 4 had cerebrospinal fluid rhinorrhea, and 3 developed a nasal obstruction. Five defects originated at the foramen cecum, and 3 others involved the ethmoid roof/cribriform plate only. Our endoscopic approaches were successful on the first attempt, with a mean follow-up of 19 months. One patient experienced nasal stenosis postoperatively. Conclusions: Continuing progress in the surgical management of congenital skull base defects demonstrates that endoscopic repair is a successful alternative to traditional craniotomy approaches, with less morbidity. This technique requires meticulous preparation and precise grafting of the defect to avoid collateral damage to surrounding structures. While reduction in the risk of meningitis, intracranial complications, and facial growth abnormalities and alleviation of nasal obstruction necessitate the timely repair of these skull base defects, special considerations are discussed regarding the optimal timing of surgical intervention, operative working space, and exposure in a smaller nasal cavity.