Neoplastic meningitis is a diffuse dissemination of tumor cells into the cerebrospinal fluid (CSF) and/or leptomeninges. It occurs in approximately 5-10% of malignant diseases, most often in breast cancer, lung cancer, melanoma or B-cell lymphoma. Symptoms of neoplastic meningitis are head or back pain, cranial nerve palsies, diffuse radicular symptoms or psychiatric disturbances. MRI shows nodular contrast enhancement lining CSF spaces. Positive CSF cytology requires optimal sampling and processing. Treatment must be individually shaped: the CSF dissemination may be treated with intrathecal chemotherapy with methotrexate or cytarabinoside (Ara-C). Liposomal Ara-C is distributed over the entire CSF space even after lumbar application and maintains cytotoxic levels for at least 2 weeks. 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.