Neural stem cells (NSCs) give rise to the entire nervous system. Animal models suggest that defects in NSC proliferation and differentiation contribute to several brain disorders (e.g., microcephaly, macrocephaly, autism, schizophrenia, and Huntington's disease). However, animal models of such diseases do not fully recapitulate all disease-related phenotypes because of substantial differences in brain development between rodents and humans. Therefore, additional human-based evidence is required to understand the mechanisms that are involved in the development of neurological diseases that result from human NSC (hNSC) dysfunction. Human-induced pluripotent stem cells provide a new model to investigate the contribution of hNSCs to various neurological pathologies. In this chapter, we review the role of hNSCs in both neurodevelopment- and neurodegeneration-related human brain pathologies, with an emphasis on recent evidence that has been obtained using embryonic stem cell- or induced pluripotent stem cell-derived hNSCs and progenitors.