Ewing's / peripheral primitive neuroectodermal tumor (pPNET) of the cavernous sinus are extremely uncommon. We present clinical, radiological, pathological findings of Ewing's / pPNET involving the cavernous sinus in an eleven-month-old infant presenting with proptosis and 6th nerve palsy. MRI Contrast scans showed a well-defined, homogenously enhancing mass involving the right cavernous sinus location. Histopathology and immunohistochemical features were consistent with Ewing's/pPNET. The patient succumbed to disease in spite of starting chemotherapy. The clinical, radiological and pathological findings of previously reported cases of Ewing's sarcoma / pPNET involving the cavernous sinus are summarized. The histological differential diagnosis of primary intracranial Ewing's sarcoma / pPNET are discussed.