Choroid plexus papillomas (CPP) are commonly seen in the supratentorial compartment in children and only very rarely in the posterior fossa. CPP in the cerebello-pontine angle and cerebello-medullary fissure (CPA) in the pediatric age group are extremely rare with only seven previous cases reported in literature. The authors present the case of a 7-year-old girl who presented with neck tilt, imbalance, and headache. The MRI showed a lesion with a frond-like appearance in the CPA with heterogeneous enhancement. The tumor was excised radically using a cerebello-medullary fissure approach. The authors review and analyze the literature on this rare pediatric tumor, with specific attention to radiology and the surgical aspects.
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