Clinical involvement of the nervous system occurs in about 5% of patients with sarcoidosis. We describe a fatal case of a young patient with neurosarcoidosis with a relatively rare psychotic syndrome in the course of neurosarcoidosis, presenting itself as a depressive syndrome with delusions. The neurological manifestations consisted of cerebellar symptoms, peripheral neuropathy and general epileptic seizures. Cerebrospinal fluid examination, serum angiotensin-converting enzyme level, magnetic resonance imaging, chest radiography, gallium isotope scanning and other tests were used as diagnostic tools. He was treated with steroids, methotrexate and neuroleptics ineffectively. The patient died because of complications related to neurosarcoidosis. The diagnosis of neurosarcoidosis was confirmed by autopsy.