Spina bifida (SB) and hydrocephalus (HC) are two distinct disorders with varying etiology but with common pathology and shared clinical and neuropsychological characteristics. Although there are now good medical interventions for children with SB and HC, there are usually significant cognitive, emotional, and behavioral consequences. While these effects are widespread and varied, there is often a characteristic pattern of strengths and weaknesses and when these are properly assessed with carefully selected neuropsychological measures and then sensitively communicated, there is good potential for targeted remediation and support, although further research is needed to identify the specific efficacy of individual strategies. With the exception of SB occulta, probably the most common and certainly the most debilitating form of SB is SB meningomyelocele, in which there is an open lesion and herniation of the spinal cord. Dennis et al. (2005) describe this as the most common severely disabling birth defect in North America, quoting a prevalence of 0.5 per 1,000 births. In fact, prevalence estimates vary hugely with geography and time. For example, Mitchell et al. (2004) report rates of 0.31 per 1,000 births in England and Wales in 1996 compared with 2.92 per 1,000 births in Northern China in the early 1990s. Rates have decreased dramatically since the 1970s thanks to increased knowledge and action regarding prenatal nutrition, but of course this is also in line with the advent of prenatal screening. Of those children who are born with SB meningomyelocele, 90-95% will also suffer from HC and the majority will have significant cognitive deficits. As such, it is this group who will be the focus of attention for the remaining sections of this chapter.