Advances in perinatal and neonatal care over the last 30 years have contributed to improved survival among extremely low birth weight (ELBW) infants. As the rate of neonatal morbidity has remained stable, parents and health care professionals involved in the care of these infants often raise questions regarding the prevalence of adverse neurodevelopmental outcomes. Existing data in the literature is heterogeneous and the reported prevalence of disability varies more than survival rates do. One of the key issues toward a better clinical management of ELBW infants is the knowledge of the nature of mortality and disability in this population of infants. Studies from individual centers report outcomes of ELBW infants and demonstrate several limitations as to the ways babies were treated. Advice to parents and decisions to be made regarding the care of these infants should be based on reliable, unbiased, and representative data drawn from geographically defined populations. Such data have recently become available. This report gives an overview of existing literature on this issue.