Meningiomas are common lesions in adults but unusual in infancy and meningiomas located in the posterior cranial fossa are even more rare. Metaplastic changes of meningothelial meningiomas can lead to the rarely observed xanthomatous form. We describe the case of a posterior pyramid xanthomatous meningioma in a 2-year-old girl. After detailed neuroradiological 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.