Purpose: To present a case of malignant peripheral nerve sheath tumour (MPNST) metastatic to the orbit.
Method/results: The authors describe a 29-year-old woman with disseminated MPNST who presented with acute right orbital inflammation. Computerized tomography revealed a superolateral mass with a central radiolucent area. At biopsy, the mass was primarily subperiosteal and histology showed a spindle cell, S100-positive MPNST consistent with the primary tumour.
Conclusion: This is the second reported case of MPNST metastatic to the orbit and illustrates that an inflammatory presentation is possible in this aggressive malignancy.