Platelet-derived growth factor (PDGF) has long been implicated in cancer and is known to be involved in many biological processes. In Central Nervous System (CNS) neoplasms, particularly gliomas, PDGF is often over-expressed. However, what role PDGF plays in tumor progression remains to be fully described. A wide range of work from in vitro studies to mouse models have implicated the PDGF pathway in various processes including proliferation, cellular migration, development, and angiogenesis. Being a secreted factor, PDGF not only has autocrine effects on producing cells but also has potential for paracrine effects on other tumor cells and the tumor microenvironment. The development of small molecules that inhibit the PDGF receptor and various subsequent signaling components promises to introduce new approaches to the treatment of gliomas.