Although helminth infections of the central nervous system (CNS) are rare, their public health implications must not be neglected. Indeed, several helminth species can cause cerebrospinal infections, especially if humans serve as intermediate or non-permissive host. The diagnosis of cerebrospinal helminthiases is difficult, and the detection of parasites in cerebrospinal fluid is rarely successful. Cerebrospinal helminth infections therefore often remain undetected, and hence prognosis is poor. Increases in tourism and population movements are risk factors for cerebrospinal helminthiases and infections pose particular challenges to clinicians in non-endemic areas. In this review, we focus primarily on food-borne helminthiases that are endemic and often emerging in Southeast Asia and the Far East, namely angiostrongyliasis, gnathostomiasis, sparganosis, paragonimiasis and cysticercosis. Additionally, we discuss neuroschistosomiasis, a disease that is transmitted through human-water contact. For each disease, we describe the pathogen, its transmission route and possible mechanisms for entering the CNS. We also summarise common signs and symptoms, challenges and opportunities for diagnosis, treatment, clinical management, geographical distribution and epidemiology. The adoption of a comprehensive set of diagnostic criteria for different cerebrospinal helminthiases is proposed, including epidemiological history, typical signs and symptoms, neuroimaging and laboratory findings. Finally, risk factors, and research needs for enhanced patient management and population-based control measures are discussed.