Neoplastic meningitis is a diffuse dissemination of tumour cells in the cerebrospinal fluid (CSF), leptomeninges, or both. It occurs in approximately 5-10% of malignant diseases, most often in breast cancer, lung cancer, melanoma, and B-cell lymphoma. Symptoms of neoplastic meningitis include head or back pain, cranial nerve palsies, diffuse radicular symptoms, and psychiatric disturbances. Magnetic resonance imaging shows nodular contrast enhancement lining the CSF spaces. Positive CSF cytology requires optimal sampling and processing, and the treatment of neoplastic meningitis must be individualized. The CSF dissemination can be treated with intrathecal chemotherapy with methotrexate or Ara-C. Radiotherapy should be applied only to symptomatic solid spinal manifestations or fast progressing cranial nerve palsies. Systemic chemotherapy is needed to control solid manifestations or, in the case of substances entering the CSF, to support intrathecal chemotherapy.