This book provides a comprehensive overview of the development of the human central nervous system (CNS) in the context of its many developmental disorders due to genetic, environmental and hypoxic/ischaemic causes. The book contains three general, introductory chapters in which an overview of the development of the human brain and spinal cord, a summary of mechanisms of development as obtained in experimental studies in various invertebrates and vertebrates, and an overview of the causes of congenital malformations are presented. The developmental disorders of the human brain and spinal cord are presented in a regional, more or less segmental way, starting with neurulation and neural tube defects, and ending with developmental disorders of the cerebral cortex. These chapters are abundantly illustrated in colour with carefully chosen clinical case studies with imaging data, and when available, postmortem verification of the developmental disorders involved.