ABSTRACT.Purpose: This review emphasizes the importance of neuro-ophthalmological signs and symptoms in sarcoidosis. The presence of ophthalmological and neuro-ophthalmological findings may lead to diagnosis of the disease and the initiation of adequate treatment. Material and Methods: Patients who had been diagnosed with neurosarcoidosis during the period 1990 À 2001 were identified from the departmental diagnostic index. The history, clinical, laboratory and imaging data of patients were analysed. Results: Fifteen patients were identified, four men and 11 women, with a mean age of 44.1 years (range 26-65 years). In six of the 15 (40%), neurological deficits were the initial symptoms. Nine (60%) had known sarcoidosis at the time of presentation. Ten patients (66%) had ophthalmological/neuro-ophthalmological symptoms and signs. Conclusion: Neuro-ophthalmological symptoms may develop early in neurosarcoidosis. If neuro-ophthalmological symptoms arise in patients with established biopsyproven sarcoidosis, the diagnosis is usually easy to make. However, a number of patients with neurosarcoidosis may present with neuro-ophthalmic symptoms before systemic involvement becomes obvious. In this situation the diagnosis is challenging, and the major goal is to establish the presence of systemic sarcoidosis.