A 35-year-old man presented with the clinical picture of spontaneous subarachnoid hemorrhage. Four weeks after the first symptoms he noticed blurred vision, and ophthalmological examination detected bitemporal hemianopia. At this time cerebral computed tomography and magnetic resonance imaging showed enlargement of the optic chiasm, and visual evoked potentials revealed delayed latencies. In the cerebrospinal fluid cells and protein content were elevated, and angiotensin-converting enzyme was detectable. Under steroid treatment the patient recovered completely and computed tomography, magnetic resonance imaging, visual evoked potentials, and cerebrospinal fluid findings became normal. Although a great variety of neurological symptoms may occur in neurosarcoidosis, to our knowledge spontaneous subarachnoid hemorrhage as the presenting feature has never been reported before.