Background Neoplastic meningitis describes diffuse subarachnoidal dissemination of tumor cells. The frequency of neoplastic meningitis is underestimated, and it can occur in almost any tumor entity. Often, neoplastic meningitis is associated with a very poor prognosis. However, numerous examples show that patients can benefit from well-planned therapy in terms of improved quality and quantity of life. Objective An overview of diagnostic and therapeutic options in the management of neoplastic meningitis is provided. Materials and methods The results of a MEDLINE search and the German guidelines are summarized. Results For diagnostic confirmation of neoplastic meningitis, a physical examination, analysis of cerebrospinal fluid and magnet resonance imaging are recommended. Pathophysiologically, new preclinical approaches like the involvement of the complement system in meningeosis development are promising. As causal therapy, approaches like radiotherapy, systemic chemotherapy and intrathecal chemotherapy are available. Symptomatic treatment options, e.g., implantation of a ventriculoperitoneal shunt or a systemic treatment with dexamethasone, are also available. The most common substances that have been used intrathecally for many years include cytarabine, methotrexate, and thiotepa, but without significant proof of efficacy. New substances targeting molecular alterations are increasingly discussed. Depending on the molecular profile of the primary tumor, osimertinib, lorlatinib, alectinib, trastuzumab-emtansine and the combination of nivolumab and ipilimumab are CNS-effective substances of interest for example. Conclusion Neoplastic meningitis remains a challenging entity. Newer approaches give hope to improve patients' quality of life but further research regarding efficacy from clinical trials is needed.