In the last 10 years, enormous interest in neural stem cells has arisen from both basic and medical points of view. The discovery of neurogenesis in the adult brain has opened our imagination to consider novel strategies for the treatment of neurodegenerative diseases. Characterization of neurogenesis during development plays a fundamental role for the rational design of therapeutic procedures. In the present review, we describe recent progress in the characterization of embryo and adult neural stem cells (NSCs). We emphasize studies directed to determine the in vivo and in vitro differentiation potential of different NSC populations and the influence of the surrounding environment on NSC-specific differentiation. From a different perspective, the fact that NSCs and progenitors continuously proliferate and differentiate in some areas of the adult brain force us to ask how this process can be affected in neurodegenerative diseases. We propose that both abnormal cell death activation and decreased natural neuronal regeneration can contribute to the neuronal loss associated with aging, and perhaps even with that occurring in some neurodegenerative diseases. Furthermore, although NSC activation can be useful to treat neurodegenerative diseases, uncontrolled NSC proliferation, survival, and/or differentiation could cause tumorigenesis in the brain. NSC-mediated therapeutic procedures must take into account this latter possibility.