Juvenile psammomatoid ossifying fibroma (JPOF) is a benign fibro-osseous lesion typically associated with the jaw, paranasal region, or orbit. However, JPOF may also originate from the skull base and locally invade the cranium. In published reports, intracranial JPOFs constitute only a small percentage of cases, and therefore it is not known whether more aggressive behavior typifies this distinct population of JPOFs compared with those in other locations. Nevertheless, JPOF histopathology is characterized by a number of active processes, including cystic transformation, that may precipitate violation of skull base boundaries. In the following article, the authors present a case of skull base JPOF that underwent cystic expansion in a young girl, produced a focal neurological deficit, and was resolved using a staged surgical approach.