We report on a patient with choriocarcinoma in the pineal region who was successfully treated with stereotactic radiation therapy (SRT). The increased level of serum human chorionic gonadotropin (HCG) was lowered during chemotherapy with etoposide, cisplatin, and ifosfamide. However, HCG was not normalized and magnetic resonance images still showed an enhanced tumor mass with gadolinium. The patient underwent SRT of 40 Gy at an 80% isodose line per 10 fractions over two weeks, followed by conventional craniospinal irradiation of 32.4 Gy. The level of HCG dropped below the detection limit. The patient has been in good condition for more than four years after the completion of treatment, without any signs of recurrence. We propose SRT as a valid treatment option for malignant germ cell tumors in the pineal region.