Astrocyte elevated gene 1 (AEG-1), a novel gene that was cloned in 2002, has emerged in recent years as a potentially crucial mediator of tumor malignancy and aberrant elevation of AEG-1 expression frequently occurs in several human cancers, including breast cancer, prostate cancer, gastric cancer. However, whether AEG-1 deregulation also occurs in neuroblastoma remains unclear. In previous study we reported that AEG-1 was over expressed in neuroblastoma cell lines and knockdown of AEG-1 inhibits proliferation and enhancing chemo-sensitivity to cisplatin or doxorubicin in neuroblastoma cells. In this study, we investigated the expression of AEG-1 and evaluate its prognostic significance by correlating AEG-1 expression levels with clinic pathologic features and survival in 32 archived neuroblastoma patients We found that positive AEG-1 immunoreactivities were present in all neuroblastoma cases, 75% showed high expression of AEG-1. And high expression of AEG-1 was commonly seen in vascular endothelial cells and glandula in neuroblastoma samples. AEG-1 expression was strongly correlated with age at diagnosis (P=0.012), clinical stage (P=0.030) and tumor histology stage (P=0.041). However, our analyses did not show significant associations between AEG-1 expression and other clinical features including gender and primary tumor site. Importantly, our data presented in this report provide, for the first time, evidence that elevated expression of AEG-1 protein is correlated with poor prognosis and reduced survival of patients with neuroblastoma (P= 0.031). Overall, the data support the notion that AEG-1 might be used as a biomarker for neuroblastoma patients.