A 60-year-old woman presenting with a 15-month history of Jacksonian seizures was found on computed tomography (CT) scan to have an enhancing subcortical lesion high in the left anterior parietal lobe. The excised tumor had light microscopic features similar to those of an epithelioid hemangioendothelioma, and immunohistochemi-cal stains confirmed its vascular nature. At surgery, the patient had no clinical or radiological (including CT body scan) evidence of any other lesion. However, 22 months after symptoms first appeared, she presented with dyspnea and bilateral pleural effusions, and a left atrial myxoma was detected echocardiographically. The histologic features of the resected myxoma were identical to those of the previously excised cerebral lesion, and it became apparent that the intracranial tumor was a metastasis of the atrial myxoma. This case illustrates that before a diagnosis of visceral epithelioid hemangioendothelioma is rendered, the possibility of metastatic occult cardiac myxoma should be considered. Echocardiography is warranted for such patients.