A 64 year-old patient, complained of headache and neurological disorders. CT scan found a voluminous solitary tumor of the posterior part of the left cavernous sinus. Removal of tumor was followed by a rapid recurrence and by the patient's death. Histologic study found a malignant undifferentiated tumoral proliferation, with strap-like cells. Immunohistochemical stains were positive for conjunctival and muscular differentiation. Ultrastructural study revealed intracytoplasmic filamentous striated structure. The primary meningeal rhabdomyosarcoma is an exceptional tumor, generally affecting young patients. Its prognosis is poor and its histogenesis remains unclear.