Neurocysticercosis is an endemic disease in Latin America, Asia and Africa with growing occurrence in industrialized countries due to the increase in migration from low- and middle-income to high-income countries. The most severe clinical presentation is when the parasite is located in the subarachnoid space at the base of the brain (NCSAB). Aside from its clinical presentation, the severity of this form of the disease is due to the difficulties in diagnosis and treatment. Although NCSAB frequency is lower than that reported for the parenchymal location of the parasite, its clinical relevance must be emphasized. We provide a critical review of the central epidemiological, clinical, diagnostic and therapeutic features of this particular form of the disease, which is still associated with unacceptably high rates of morbidity and mortality.