Patients diagnosed with intracranial teratoma are at risk for developing a recurrent malignant germ cell tumor. We describe a 14-year-old boy initially diagnosed with a mature teratoma in the pineal region that recurred as a metastatic beta-human chorionic gonadotropin (betaHCG)-secreting germ cell tumor 3 years after gross total resection. A surveillance brain MRI scan during follow-up demonstrated multiple lesions within the ventricular and subependymal area infiltrating the brain parenchyma along with concomitant elevated levels of betaHCG in both the serum and cerebrospinal fluid. The patient underwent chemotherapy with PEI (cis-platinum, etoposide, ifosfamide) followed by radiation therapy according to the SIOP CNS GCT protocol. The patient is currently alive without evidence of disease 35 months after starting therapy. CONCLUSIONS: A careful and long-term follow-up including scheduled tumor markers as well as surveillance MRI scans is required for patients with intracranial teratoma in an effort to detect and diagnose recurrent malignant disease, especially since multimodal therapy provides the potential for long-term cure.