Primary central nervous system (CNS) teratomas are rare tumors that consist of all three germ cell layers. We describe a young man with a primary malignant CNS teratocarcinoma presenting as leptomeningeal carcinomatosis. Diagnosis of primary CNS teratocarcinomas is challenging; relentless pursuit of the diagnosis must follow even if early ancillary studies are inconclusive.
Keywords: central nervous system; leptomeningeal malignancy; teratocarcinoma.