The progamme of cell death called apoptosis is a biochemical and genetical pathway which has been well conserved throughout evolution. In the past years, the involvement of apoptosis has been shown not only during the embryonic development of the nervous system, but also in neurodegenerative diseases. Moreover, resistance to apoptosis is clearly an important factor in tumor growth and seems to be, at least partially, a major process in chemo- and radio-resistance. In this review, we briefly describe the main molecular events of the apoptotic process, known to date, and discuss possible defects in the central nervous system diseases.