In order to gain greater insight into the molecular basis of neurological disease, scientists have been restricted primarily to genetically malleable animal models. However, for a number of reasons including genetic differences between humans and animals, the field has needed additional systems to better recapitulate the human condition. In this chapter we will review the use of human pluripotent stem cells as an unlimited source of affected tissue that can be used to model disease. In addition, we will discuss how these models may allow us to better understand the mechanism of these diseases as well as influence the generation of treatments.