INTRODUCTION Glioblastoma multiforme (GBM) is a very rare type of tumor involving the pineal region. No case of pineal region GBM with tumoral hemorrhage has ever been reported. Here, we present the first such case report. CASE REPORT A 65-year-old man presented with sudden-onset disturbance of consciousness. CT revealed intraventricular hemorrhage and acute hydrocephalus. The patient was treated by an emergent operation for ventricular drainage. MRI with contrast media showed a heterogeneous enhanced area in the pineal region. The patient underwent endoscopic biopsy surgery and a third ventriculostomy for non-communicating hydrocephalus. The pathological diagnosis was high-grade glioma, indicating the need for tumor removal via the occipital transtentorial approach. The tumor was removed subtotally and radiation and chemotherapy courses were then started. CONCLUSION Pineal GBM with tumoral hemorrhage has never been reported before. Endoscopic operations are useful for rapid decision making prior to radical surgery.