Myelomeningocele, a form of spina bifida where the vertebrae fail to fuse during foetal development, is often associated with sensory deficits, reduced motor function and hydrocephalus. These complications may interfere with the development of the central nervous system affecting myelination of axons and formation of synaptic connections, and development of cognitive skills, particularly where complications occur within the first twelve months of life. This paper examines the long-term cognitive outcome in nineteen children with myelomeningocele, who were shunted for hydrocephalus within the first year of life. In comparison to a healthy control group matched for age and gender, the clinical sample were found to have globally compromised cognitive skills, including reduced intellectual and educational skills. Specific impairments were noted on measures of speed of processing, immediate registration of information, learning and memory, organisation and high level language, with greatest deficits on more complex, less structured tasks. Within the clinical group, there was a fall in IQ over time, suggesting a failure to acquire cognitive skills in the expected time frame. These results, reflecting a cumulative pattern of deficits over time, highlight the importance of long-term follow lip in children with myelomeningocele and perinatal hydrocephalus.