Conclusion: Primary intracranial myxoma should be distinguished from other myxoid intracranial tumors such as myxomatous meningioma, epithelioid hemangioendothelioma or sarcoma through appropriate pathological and inmunohistochemical analysis. A metastasic cardiac myxoma should also be ruled out through cardiac evaluation including an echocardiography. The distinction between this entity and the reported neurothekeoma of the meninges needs to be reevaluated.