Isolated skull base primary Ewing's sarcoma: an extremely rare location.

A 16-year-old boy presented with history of headache and blurring of distant vision. He had UMN facial paresis and papilledema. Imaging revealed a heterogenous space occupying lesion in the middle cranial fossa base with mass effect and midline shift and non homogenous contrast enhancement with erosion of the petrous bone. He underwent gross total excision of the lesion. Histopathology and immunohistochemistry were suggestive of skull base Ewing's sarcoma. Bone scan was done to rule out primary involvement of other extracranial location. We have discussed the epidemiology, presentation, management and prognosis of such cases.