A l7-year-old girl presented with fever and headache. A brain CT found tentorial thickening that mimicked tuberculous meningitis. She had received antituberculous medication, which failed to respond, and subsequently developed anemia, hepato-plenomegaly and lymph- adenopathy. A bone marrow aspiration revealed erythrophagocytic histiocytes and a lymph node biopsy disclosed lysozyme positive malignant histiocytes. She died 7 months after the onset of the illness. We report a case of malignant histiocytosis involving meninges at initial presentation.