Objective: Pseudotumor cerebri syndrome (PCS) is characterized by increased intracranial pressure without the presence of a lesion. Rarely diagnosed in children under the age of 11, knowledge of PCS-related cognitive deficits has largely been gathered from adult samples. The current examination will add to the limited information on pediatric presentations. Method: The patient is a Caucasian female, first diagnosed with PCS at age 9. She has common comorbid diagnoses including strabismus, optic nerve drusen, papilledema, and had a lumboperitoneal shunt placed at age 10. Neuropsychological complaints include poor memory for daily routines and difficulty in mathematics and reading comprehension. Results: Results of testing showed borderline full scale IQ (FSIQ = 76). Although the patient showed mastery of basic academic skills such as word reading and arithmetic, she struggled with comprehension and application. Memory for visual and verbal information was impaired, and while basic visual-perceptual skills were intact, more advanced visual-perception, visual-construction, and motor coordination were impaired. The patient's receptive and expressive language skills were low average, while abilities for word fluency and advanced visual naming were impaired. Conclusion: Deficits with visual perception, construction, and motor coordination were expected, given the patient's visual difficulties, and impaired FSIQ was consistent with previous adult studies. Importantly, deficits were not localized to a particular region, but rather demonstrated a diffuse pattern of impairment. Our results challenge the belief PCS is a “benign” disorder and supports the role of routine neuropsychological evaluation in children with this disorder. Evaluation and remediation may help increase functional aspects of life for these patients.