A 28 year-old man complaining of intermittent headaches for a month following an episode of general convulsions was admitted to the Department of Neurosurgery of our hospital. His brain CT scan showed a well-enhanced mass growing symmetrically along the anterior cerebral falx in a manner compatible with meningioma. Nothing abnormal was detected in chest radiography on admission. Histopathological examination of the mass after partial resection revealed multiple epithelioid cell granulomas with giant cells indicating sarcoidosis. After 4 weeks of treatment with oral prednisolone 30 mg/day, bronchoalveolar lavage was performed in our department. In the lavage fluid, both the total cell count and the percentage of lymphocytes were elevated. The lung specimens obtained by transbronchial biopsy also showed epithelioid cell granulomas. CNS sarcoidosis is reportedly usually observed in the vicinity of the hypothalamus with other organ involvement. In the present case, differential diagnosis of sarcoidosis from meningioma was difficult because the solitary lesion was growing along the anterior cerebral falx without radiographic evidence of bilateral hilar lymphadenopathy.