A reliable biomarker has not been identified to predict the outcome of traumatic brain injury (TBI) in children. Therefore, the present systematic review and meta-analysis aimed to assess the association between neuron specific enolase (NSE) and traumatic brain injury (TBI) in children. Two independent reviewers searched electronic databases of EMBASE, Cochrane library, Medline and Scopus and then they summarized the results and did a quality control check. At the end, standardized mean difference (SMD) with 95 % confidence interval (CI) and performance of NSE were assessed. 10 studies were included in the present meta-analysis. Average serum (SMD=1.3; 95 % CI: 0.5 to 2.1; p=0.001) and CSF levels (SMD=2.45; 95 % CI: 1.04 to 3.8; p<0.0001) of NSE biomarker were significantly higher in children with TBI with unfavorable outcome compared with other children. Serum NSE had an area under the curve, sensitivity and specificity of 0.75 (95 % CI: 0.72 to 0.79), 0.74 (95 % CI: 0.64 to 0.82) and 0.69 (95 % CI: 0.59 to 0.77), respectively in prediction outcome of TBI. Positive likelihood ratio, negative likelihood ratio and diagnostic odds ratio of serum NSE were 2.4 (95 % CI: 1.7 to 3.3), 0.38 (95 % CI: 0.26 to 0.55) and 6.0 (95 % CI: 3.0 to 12.0), respectively. The results show that the performance of NSE is in a moderate level in prediction of unfavorable outcome in children with TBI. However, data in this aspect is not sufficient and more studies are needed.