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Abstract: Background: Only four cases of primary intracerebellar paragangliomas have been reported in the literature to date. Because of its rarity, primary intracerebellar paraganglioma still presents a diagnostic challenge for both radiologists and neurosurgeons, and the optimal therapeutic modality is still debatable for its hypervascularity and location. Patients: We report a 16-year-old boy with pathology-proven primary intracerebellar paraganglioma who presented with dull headache, dizziness, and gait disturbance, and underwent gross total resection. Further, we review all reported cases of primary intracerebellar paraganglioma in the English literature and discuss its clinical profile, neuroradiological features, and treatment modalities. Results: His symptoms improved following tumor removal without radiotherapy, and postoperative neuroimaging thirteenth months after surgery showed no recurrence. In the literature, all four patients were stable in the follow-up period including three with complete resection and one with partial resection plus adjuvant radiotherapy. Conclusion: Surgical resection is the treatment modality most often used for primary intracerebellar paraganglioma; radiation therapy may be used when there is residual tumor or recurrence. Angiography may help to clarify the vessel architecture for reducing intraoperative bleeding when primary intracerebellar paraganglioma is considered. [Copyright &y& Elsevier]