Abstract
BACKGROUND: Pineal region hemangioblastoma(PR-HB) is an exceptionally rare vascular tumor, often misdiagnosed due to overlap with more common pineal lesions. Accurate identification is essential for safe surgical management. CASE PRESENTATION: We report a 54-year-old woman presenting with acute dizziness, nausea, and vomiting. MRI revealed a vividly enhancing pineal mass with prominent flow voids causing obstructive hydrocephalus. The tumor was removed via a left paramedian supracerebellar infratentorial approach, achieving gross-total resection. Histopathology and immunohistochemistry confirmed hemangioblastoma (inhibin-α positive, PAX8 negative). The patient recovered uneventfully, and no recurrence was observed at 12-month follow-up. DISCUSSION: Although extremely rare, pineal hemangioblastoma should be considered in the differential diagnosis of hypervascular pineal masses. Review of previously published cases demonstrates consistent radiological features, diagnostic pitfalls, surgical considerations, and the importance of distinguishing hemangioblastoma from metastatic renal cell carcinoma. CONCLUSION: Pineal hemangioblastoma is a surgically curable entity. Recognition of its imaging and immunohistochemical characteristics facilitates safe resection and accurate diagnosis.