BACKGROUND‐Nervous system manifestation of sarcoidosis (neurosarcoidosis) may occur in 5 to 10% of patients who have systemic sarcoidosis. Neurosarcoidosis may also occur in patients who have not had a previous diagnosis of sarcoidosis (primary neurosarcoidosis). We discuss the clinical presentation of primary neurosarcoidosis, treatment response, and outcome in this study.REVIEW SUMMARY‐Records of 22 patients with neurosarcoidosis who were not known to have systemic sarcoidosis at the time of neurologic presentation were reviewed. The clinical presentation and diagnostic procedures that led to the diagnosis in these patients are summarized. Neurologic presentations were highly variable. Neuroimaging was abnormal in all patients when contrast was used; meningeal enhancement was the most common finding. Spinal fluid was abnormal in 95% of patients. Seventy percent of chest x‐rays and 80% of chest CT scans performed at the time of presentation were abnormal. All 12 gallium scans that were performed were abnormal. Diagnosis was confirmed by biopsy of neural tissue in 36% and of non‐neural tissue in 64%. Initial response to oral corticosteroids was favorable, but more than half of the patients at 1 year had deteriorated.CONCLUSIONS‐The evaluation of patients with primary neurosarcoidosis can be optimized by combining clinical suspicion, based on presenting symptoms and signs, with spinal fluid and neuroimaging studies. Appropriate use of chest x‐ray and CT and gallium scans frequently leads to diagnosis from lymph node or lacrimal gland biopsy.(THE NEUROLOGIST 6:126‐133, 2000)