SummaryThe frequency of neurologic involvement in sarcoidosis is about 5 %. When the clinical picture presents with only neurological manifestations, as it did in our patient, the diagnosis at presentation is often difficult. Involvement of other tissues must therefore be actively searched.We describe a case of neurosarcoidosis, presenting with hypothalamic hypopituitarism, progressive loss of visual fields, subacute dementia and hypothalamic hyperphagia. The disease very well responded to high dose corticosteroids. The pathology, diagnostic procedures, prognosis and treatment of neurosarcoidosis are discussed.