Astrocyte elevated gene 1 (AEG-1), an important oncogene, has been confirmed to play a crucial role in progression (invasion, metastasis, angiogenesis and chemoresistance, etc) of multiple cancers. Over the past decade, studies have shown that AEG-1 is overexpressed in cancers involving almost all organs, including neuroblastoma, melanoma, HCC, breast, prostate cancer, etc. Moreover, AEG-1 is associated with multiple pathways including PI3K/Akt and nuclear factor-κB (NF-κB). This review mainly outlines the multiple regulating role of AEG-1 in the development and progression of tumor and to discuss its role as a novel target for treatment of malignant tumors.