Objective: The treatment of hydrocephalus in children with endoscopic third ventriculostomy (ETV) has particular features and is associated with different success rates (SR). The aim of this study was to identify putative factors that could influence the outcome of ETV in children. Methods: Clinical data of 114 consecutive patients under 18 years of age who underwent 116 consecutive ETVs from January 2000 to January 2010 were reviewed. Data were analyzed with regards to clinical and radiological SR. The actual long-term SR was compared to that predicted by the ETV Success Score (ETVSS) model. Results: The study group included 49 males (43%) and 65 females (57%) with a mean age of 6.17 ± 1.02 years (ranging from 11 days to 18 years) at surgery. Concerning the etiology of hydrocephalus, tumors and aqueductal stenosis (AS) were the most frequently observed, with each occurring in 33 cases (29%), followed by malformations in 24 (21%), cystic lesions in 6 (5%) and other etiologies in 18 patients (16%). The overall SR at the first ETV attempt was 80% (91/114), compared to 74.8% (variance 14.35, 95% CI 69.37-78.22) predicted by the ETVSS. Regarding age, SR was 58% in patients under 6 months of age, 65% in children between 6 months and 1 year, and 86% in children older than 1 year. SR for AS and hydrocephalus associated with posterior fossa tumors were 88 and 90%, respectively. Unsatisfactory results were related to previous intraventricular hemorrhage and infection. The overall complication rate in this series was 13%. Conclusion: ETV is safe and effective in children. In this series, the age of the patient and etiology of hydrocephalus were related to SR. Also, the ETVSS was accurate to predict outcome. In a long-term follow-up, surgical experience was statistically significant in reducing complications.