A case of pineocytoma associated with intraventricular and meningeal metastasis is reported. The patient, a 25-year-old female, was admitted complaining of headache. Computed tomography revealed an irregular-shaped pineal lesion enhanced by contrast medium and accompanied by a cyst. An intraventricular cystic metastatic lesion and meningeal metastasis were also suggested. Biopsy of the pineal region proved the lesion to be a pineocytoma without neuronal or glial differentiation. Ventriculoperitoneal shunting was performed, and radiation therapy combined with chemotherapy (ACNU and vincristine) was administered. This treatment apparently destroyed both the primary and metastatic lesions, and the patient returned to her normal life. In 4 years of follow-up there has been no recurrence of the tumor. Pineocytoma with meningeal metastasis usually has a poor prognosis, but in this case combined radiation therapy and chemotherapy was curative. Although pineocytoma is rarely accompanied by a cyst, in cases of a pineal lesion coexistent with a cyst, a diagnosis of pineocytoma should be considered.