Inflammatory pseudotumor (IP) is a benign process that most commonly occurs in the lung and orbit. Extension into the central nervous system is extremely rare, and primary intraventricular lesions of the lateral ventricles are even more infrequent with only 2 cases reported in pediatric patients to date. Here, the authors present an unusual case of IP occurring in a 16-year-old female presenting with a 2-week history of progressive headaches and vomiting, without focal neurological deficits or radiographic evidence of hydrocephalus. The patient underwent left parietal craniotomy and complete resection of the tumor, with no signs of recurrence at 3-month follow-up. Although the rarity of intraventricular IP in pediatric patients can make its initial identification difficult, IP should be considered as a potential diagnosis in this population wherein good outcomes may be achieved following surgical resection.