For the past decade, DISC1 has been studied as a promising lead to understand the biology underlying major mental illnesses, such as schizophrenia. Consequently, many review articles on DISC1 have been published. In this article, rather than repeating comprehensive overviews of research articles, we will introduce the utility of DISC1 in the study of cortical development in association with a wide range of developmental brain disorders. Cortical development involves cell autonomous and cell nonautonomous mechanisms as well as host responses to environmental factors, all of which involve DISC1 function. Thus, we will discuss the significance of DISC1 in forming an overall understanding of multiple mechanisms that orchestrate corticogenesis and can serve as therapeutic targets in diseases caused by abnormal cortical development.