Neurocysticercosis (NCC), the most common central nervous system (CNS) parasitic infection among the immunocompetent population can imitate every clinical feature of brain-diseases accurately, drawing attention away from the real culprit and delaying the proper treatment. There are two types of NCC, the parenchymal and the extraparenchymal form. The extraparenchymal NCC include the ventricular cysticercosis, the subarachnoid cysts including giant cysts or racemose cysticercosis with chronic meningitis, the spinal (intra- or extramedullary) cysticercosis and the ophthalmic cysticercosis. It is estimated that about 30% of epilepsy cases in endemic countries are due to NCC and especially the racemose NCC is more aggressive and associated with higher mortality rates. There is a significant heterogeneity in clinical phenotypes, regarding the racemose NCC, which depends on the parasite load and evolutionary stage in association with its location in CNS and the host’s immune response. Crucial for the management of the racemose NCC is the early recognition of the symptoms and the swift initiation of antiparasitic therapy with anti-inflammatory agents in combination with the shunt-insertion in cases of obstructive hydrocephalus. In view of the former considerations we conducted a narrative literature review on racemose NCC and described the diagnostic challenges of a relevant case that we had evaluated in our Department of Neurology.