Neural cell death has a pivotal role in both the development and pathophysiology of the nervous system. Two distinct modes of cell death—necrosis and apoptosis—are involved in pathological neuronal loss, but apoptosis alone is the mechanism of programmed cell death during development. All cells will undergo apoptosis in the absence of survival signals, usually peptide growth factors secreted by other cells. This provides an elegant mechanism for the control of neuronal development: a surplus of neurons is produced, and only those that form the correct connections with the target tissue receive adequate survival factors. The remainder undergo apoptotic death and removal. Apoptosis continues throughout life and is the central mechanism for the removal of surplus, unwanted, damaged or aged cells. Dysregulation of apoptosis is seen after cellular insults or in neurodegeneration as well as in tumourigenesis. Strategies which influence the apoptotic pathway offer valuable therapeutic approaches in a variety of pathological states.