Helminth infections are common worldwide and include infections with roundworms (nematodes) and flatworms (cestodes and trematodes). Infections of the central nervous system (CNS) can be caused by a wide range of helminths. As a group, they are common in resource-limited countries, but are rare in wealthy countries. Among these infections, neurocysticercosis is an important cause of seizures worldwide and also a cause of hydrocephalus. Angiostrongylus, Baylisascaris, Toxocara, and Gnathostoma among others can cause eosinophilic meningitis. In this chapter we review epidemiology, presentation, diagnosis, treatment and prevention of the common helminthic infections including infections caused by cestodes, nematodes and trematodes.