Spinal cord development occurs through three consecutive periods. Gastrulation (weeks 2-3) is characterized by conversion of the embryonic disk from a bilaminar to a trilaminar arrangement and establishment of a notochord. Primary neurulation (weeks 3-4) produces the uppermost nine tenths of the spinal cord. Secondary neurulation and retrogressive differentiation (weeks 5-6) result in formation of the conus tip and filum terminale. Defects in these early embryonic stages produce spinal dysraphisms, which are characterized by anomalous differentiation and fusion of dorsal midline structures. Spinal dysraphisms may be categorized clinically into two subsets. In open spinal dysraphisms, the placode (non-neurulated neural tissue) is exposed to the environment. These disorders include myelomeningocele, myeloschisis, hemimyelomeningocele, and hemimyelocele, and are always associated with a Chiari II malformation. Closed spinal dysraphisms are covered by intact skin, although cutaneous stigmata usually indicate their presence. Two subsets may be identified based on whether a subcutaneous mass is present in the low back. Closed spinal dysraphisms with mass comprise lipomyeloschisis, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise complex dysraphic states (ranging from complete dorsal enteric fistula to neurenteric cysts, split cord malformations, dermal sinuses, caudal regression, and spinal segmental dysgenesis), bony spina bifida, tight filum terminale, filar and intradural lipomas, and persistent terminal ventricle. Magnetic resonance imaging is the imaging method of choice for investigation of this complex group of disorders.