Background: Inflammatory pseudotumor of the orbit is a relatively rare, benign, non-infective inflammatory process with no known local or systemic causes. Extension into the intracranial compartment
is extremely uncommon.Case Report: We present a case of a 42 year old woman with extensive intra-cranial involvement of an orbital pseudotumor. The lesion responded dramatically to therapy. The patient subsequently defaulted from clinic and represented five years later with recurrence more extensive than the initial presentation involving the internal acoustic meatus with associated hearing loss.Conclusions: We would like to highlight this uncommon lesion as it is, to the best of our knowledge, the first reported case to involve the internal acoustic meatus. A brief review of this entity, its natural history and treatment is discussed.

Keywords: orbital pseudotumor, intracranial, acoustic tumor