Gliomas are the most common primary tumors that arise from glial cells and their precursors in the central nervous system. Most of the genetic alterations identified in human gliomas result in signal transduction abnormalities or disruption of cell cycle arrest pathways. Over the past years, several mouse glioma models have been generated based on human genetic abnormalities and the induced gliomas exhibit histological similarities to their human counterparts. There is emerging evidence suggesting that an oncogenic signaling initiating tumorigenesis is also required for tumor maintenance, these glioma models can be used to further characterize the mechanisms of oncogenic signaling in tumor formation, as well as identify molecular targets in preclinical trials.