Pai syndrome is a rare congenital disorder, and there are few reports about the long-term prognosis of mental development and surgical results. Here, we report a patient with Pai syndrome who was followed up from birth up to the age of 8 years. Additionally, we review 32 articles and discuss the long-term prognosis of Pai syndrome. In our case, an intracranial lipoma grew a little, but neither epilepsy nor intellectual disabilities occurred. However, she showed attention-deficit/hyperactivity disorder. Furthermore, her nasal airway was gradually obstructed by a residual intranasal polyp.
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