A 27-year-old man was referred to our hospital because of an abnormal chest shadow. Chest unenhanced computed tomography demonstrated multiple nodules, and hilar and mediastinal lymphadenopathies. The histopathologic features of the specimens obtained from transbronchial lung biopsy showed non-caseating epithelioid cell granuloma, so we diagnosed sarcoidosis. Although the pulmonary sarcoidosis improved a year later, at that same time he experienced memory disturbance and headache. Brain contrast-enhanced magnetic resonance imaging on late-gadolinium enhancement showed hydrocephalus and nodular enhancing leptomeningeal lesions. Neuroendoscopy revealed the obstruction of the aqueduct, and endoscopic third ventriculostomy was performed. We diagnosed hydrocephalus caused by neurosarcoidosis, with meningeal biopsy showing non-caseating epithelial cell granuloma.