Background <p>The physical manifestations of craniosynostosis are well-documented, but its impact on neurodevelopment and cognitive functions remains under-researched. Evaluating neurodevelopment and cognition is essential to establish baseline cognitive functions in surgical procedures, guiding therapeutic and rehabilitation strategies post-surgery. This study aims to characterize the cognitive profile and neuropsychomotor development in individuals with craniosynostosis, identifying the most affected functions.</p> Methods <p>This study was a retrospective, observational, cross-sectional chart review that analyzed 65 individuals with craniosynostosis (39 syndromic, 26 isolated) who underwent neuropsychological assessments. Five key instruments were used to assess neuropsychomotor development and cognitive functions across different age groups.</p> Results <p>Significant neuropsychological differences were found between syndromic and isolated groups (<i>p</i> = 0.026). Key findings include the following: 68.4% of syndromic patients showed altered neuropsychological results; 40.7% of the isolated group demonstrated neuropsychological variations; language skills most frequently affected; fluid intelligence deficits in 45.5% of patients; motor gross impairments in 24.0%, predominantly in the isolated group. A weak negative correlation was observed between surgery age and cognitive improvement (<i>r</i> = −0.32), with early surgery (0–1 year), mixed outcomes, and later surgery (3 + years), poorer cognitive results.</p> Conclusion <p>Early interventions are crucial for reducing developmental delays, though individual variability and syndromic conditions significantly influence outcomes. Focusing on early intervention, targeted rehabilitation, and smooth school transition can optimize cognitive development and manage potential delays.</p>