Background: Patients with sagittal craniosynostosis are at increased risk of developing raised intracranial pressure (ICP) and neurocognitive deficiencies such as reduced attention, planning, speech, behavioural and learning disabilities. Aim: To determine if the existing literature supports a correlation between elevated ICP and negative cognitive outcome in patients with sagittal craniosynostosis. Secondly, to investigate if the risk of developing neurocognitive deficiencies can be explained by changes in brain morphology in this patient category. Methods: Systematic literature review in PubMed. Results: A total of 190 publications were reviewed to determine a possible correlation between raised ICP and cognitive outcome, of which four were included in the study. No significant association was found. Forty-four publications on brain morphology in sagittal craniosynostosis were identified, of which 11 were included in the review. Clear evidence of morphologic changes in multiple areas of the brains of sagittal craniosynostosis patients was found in the literature. Conclusion: The existing literature does not support an association between increased ICP and negative global cognitive outcome measures in patients with sagittal craniosynostosis. Brain morphology is altered in areas related to neurocognition and language in the same patient group. These changes might play a role in the development of neurocognitive deficiencies, though no definitive link is yet established, and further investigation is warranted.