A 39-year-old woman was referred to the neurology department due to headache, instability and difficulty walking for 5 months. Several ancillary tests were performed. The blood test showed leucocytosis and the cerebrospinal fluid revealed an increased total protein and glucose consumption. Other infections or autoimmune causes were excluded. The MRI showed non-specific brain and spinal cord lesions. Given the findings described, a differential diagnosis between granulomatous meningoencephalitis and primary tumour or metastasis was proposed. Empirical treatment with tuberculostatic agents and corticosteroids was started. The neurological state of the patient worsened, she fell into a non-responsive coma and died in few days. The clinical autopsy performed revealed an adenoid cystic carcinoma with involvement of the central nervous system that developed leptomeningeal dissemination along the spinal cord in a fluid 'wash' pattern.