Background. The incidence of leptomeningeal involvement from carcinomas, meningeal carcinomatosis, is underestimated and often an event of advanced stages of the disease. Objective. The incidence and mortality in patients with meningeal carcinomatosis, description of the clinical presentation, diagnostic procedures and current treatment recommendations. Material and methods. Analysis of the available literature, own observations as well as discussion of review articles and expert recommendations. Results. The great variability of clinical symptoms is because completely different sections of the central nervous system (CNS) can be affected. In many cases diagnosis is hampered by mild and uncharacteristic or even lack of clinical symptoms. All regions of the leptomeninges can be involved. To establish the diagnosis magnetic resonance scanning of the skull and the spine is as important as the cytological examination of cerebrospinal fluid. As the CNS is a sanctuary site, which is barely reached by systemic chemotherapy, treatment approaches directed at the CNS, such as intrathecal administration of cytotoxic drugs, radiotherapy and in rare cases surgical procedures such as shunt insertion can be necessary. Conclusion. The prognosis of patients with leptomeningeal disease is very poor and survival is limited to weeks up to few months mostly due to progressive systemic disease. Long-term remission or even cure is possible using combined treatment approaches only in patients with hematological malignancies, such as lymphoma and leukemia.