OBJECTIVE AND IMPORTANCE:Primary myxoma of the central nervous system is an extremely rare tumor arising from cells of primitive mesenchymal origin. Only two cases of primary intracranial myxoma have been described previously. We report a patient with a primary myxoma originating from the right frontoparietal convexity dura, which we studied in detail with diagnostic imaging and pathological analysis.CLINICAL PRESENTATION:A female adolescent presented to the emergency department with a 3-day history of mild headache, abdominal pain, and intermittent left-sided focal motor seizures. Neurological examination was remarkable for left leg hyperreflexia and difficulty with tandem gait. Cranial computed tomography and magnetic resonance imaging demonstrated an inhomogeneously enhancing mass in the right frontoparietal region.INTERVENTION:A right frontoparietal craniotomy was performed. During surgery, a tumor appearing similar to a typical convexity meningioma was completely removed along with the dural attachment.CONCLUSION:The patient had an uneventful recovery and returned to normal activity. Primary intracranial myxoma should be distinguished from other meningeal tumors and metastatic cardiac myxoma by appropriate pathological analysis and cardiac evaluation. A circumscribed myxoma completely excised with adequate dural margin carries a good prognosis for surgical cure.