Background Malignant myoepithelioma is a relatively rare malignant tumor occurring most frequent ly in the salivary glands.A few isolated cases have been described in other locations,including soft tissue, bone, lung, bronchus, oral cavity, nasopharynx, larynx, and max illary sinus.Malignant myoepithelioma, however, is uncommonly involved within the cavernous sinus.To the best of our knowledge, this is the first report of malignant myoepithelioma arising from within the cavernous sinus.Case presentation Herein, we report a case of a 48-year-old woman who presented a 1-month history of diplopia and blepharoptosis as well as radiological evidence of a rapidly developing cavernous sinus tumor.The patient underwent a trans-sphenoidal biopsy and a histo logical diagnosis indicated a malignant myoepithelioma.After diagnosis, the tumor grew rapidly and her clinical condition deteriorated progressively.Therefore, a pterion al craniotomy with partial tumor removal was performed.The patients clinical state was worsened, and she died two months after the initial operation.Because the ma lignant myoepithelioma could not be traced to an organ of origin, other than the cav ernous sinus, this case was diagnosed as a primary intracranial malignant myoepithe lioma.Conclusion The purpose of presenting this case report is to raise awareness among clinicians to consider malignant myoepithelioma as a differential diagnosis when a cavernous sinus mass is identified.Furthermore, an ideal management strategy for malignant myoep ithelioma is not known and the prognosis seems to be unfavorable; therefore, more cases are needed to enhance our knowledge of the diagnosis, treatment, and prognosis of this rare intracranial lesion.