Manifestations of an atypical variant of medulloblastoma of the posterior fossa in extra-axial locations have been reported, and key questions concerning its interpretation have been raised previously. This review illustrated the clinico-radiological and histopathological features of the posterior fossa extra-axial medulloblastoma and described possible management strategies. We thoroughly reviewed all atypical anatomical locations of medulloblastoma reported within the posterior fossa and extra-axial spaces. The main characteristics of diagnostic imaging and histopathological results, primarily the distinctive radiopathological characteristics, were summarized to distinguish between intra- and extra-axial medulloblastoma, or pathologies mimicking this tumor. Most cases of posterior fossa extra-axial medulloblastoma have been reported in the cerebellopontine angle, followed by the tentorial and lateral cerebellar locations. The dural tail sign, which is commonly observed in meningioma, is rarely seen in intra- or extra-axial medulloblastoma and might be associated with other benign or malignant lesions. In addition to magnetic resonance imaging, the proposed new imaging techniques, including advances in modern neuroimaging modalities, were discussed, as potentially efficient modalities for characterizing extra-axial medulloblastoma. Radionuclide imaging and magnetic resonance perfusion imaging are practical alternatives to limit the number of differential diagnoses. We believe that medulloblastoma cases are likely under-reported because of publication bias and frequent tumors in unusual locations. Addressing these issues would help establish a more accurate understanding of this entity.
Keywords: Cerebellopontine angle; Foramen magnum; Lateral cerebellum; Medulloblastoma; Tentorium cerebelli.