Meningiomas are common lesions in adults but unusual in infancy and me
ningiomas located in the posterior cranial fossa are even more rare. M
etaplastic changes of meningothelial meningiomas can lead to the rarel
y observed xanthomatous form. We describe the case of a posterior pyra
mid xanthomatous meningioma in a 2-year-old girl. After detailed neuro
radiological evaluation, the histological diagnosis was confirmed with
the aid of immunohistochemical evaluation. A critical case evaluation
in the light of the more recent literature, the surgical strategy and
technique, and an immunohistological hypothesis are reported.