We report two rare cases of primary choriocarcinoma in the pineal region verified histologically. In both cases, the pre-operative serum level of human chorionic gonadotropin (HCG) was significantly elevated to 128-/+935.7 and 9 -/+088.9 mIU/ml, respectively, and serum alpha-fetoprotein (AFP) was negative. The tumors were microsurgically removed, and postoperative hydrocephalus were treated by endoscopic third ventriculostomy. Both patients underwent chemotherapy and radiotherapy. After adjunctive treatment, the serum HCG decreased within normal range. During the two-year-long follow-up, no radiological (MRI) evidence was found to suggest recurrence in MR imaging, and the serum HCG was normal in one patient, but mildly elevated in the other. HCG measurement can be crucial to the diagnosis and post-treatment monitoring of choriocarcinoma, and radical surgical tumor removal and combined modality therapy including chemotherapy and radiotherapy may ensure good results.