Neoplastic meningitis is a complication of the CNS that occurs in 3-5% of patients with cancer and is characterised by multifocal neurological signs and symptoms. Diagnosis is problematic because the disease is commonly the result of pleomorphic manifestations of neoplastic meningitis and co-occurrence of disease at other sites. Useful tests to establish diagnosis and guide treatment include MRI of the brain and spine, cerebrospinal fluid (CSF) cytology, and radioisotope CSF flow studies. Assessment of the extent of disease of the CNS is of value because large-volume subarachnoid disease or CSF flow obstruction is prognostically significant. Radiotherapy is an established and beneficial treatment for patients with neoplastic meningitis with large tumour volume including parenchymal brain metastasis, sites of symptomatic disease, or CSF flow block. Because neoplastic meningitis affects the entire neuraxis, chemotherapy treatment can include intra-CSF fluid (either intraventricular or intralumbar) or systemic therapy. Most patients (>70%) with neoplastic meningitis have progressive systemic disease and consequently treatment is palliative and tumour response is of restricted durability. Furthermore, as there is no compelling evidence of a survival advantage with aggressive multimodal treatment, future trials need be done to determine the effect of treatment on quality of life and control of neurological symptoms.