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We report a case of pseudotumor cerebri associated with hematologic disorder, polycythemia vera. The patient was 35 years old woman and experienced headache and nausea for three months. She presented bilateral papilledema with visual field defect. On MRI investigation no intracranial abnomality were observed except empty sella syndrome. Lumbar puncture showed increased intracranial pressure and normal biology. Hematologic study showed elevated hemoglobin and hematocrit level. The patient was treated with therapeutic lumbar puncture and acetazolamide. Repeated phlebotomy was done for treatment of polycythemia vera, and symptoms resolved gradually. Key words:Pseudotumor cerebri;Polycythemia vera.