Taenia solium is a zoonotic tapeworm found globally but with particularly high transmission and hyperendemnicity in parts of Latin America, South and South-East Asia and sub-Saharan Africa. Depending on its life cycle, it causes two distinct presentations in humans: taeniasis and (neuro) cysticercosis. Although people with taeniasis do not have severe disease, they shed T. solium eggs, which can infect both pigs and humans. The resulting larvae form cysts in the muscles, skin, eyes or central nervous system (cysticercosis). “Neurocysticercosis” refers to the development of T. solium cysts in the human central nervous system, which causes focal epilepsy, epileptic seizures, hydrocephalus, chronic headaches, focal deficits and symptoms associated with increased intracranial hypertension. Neurocysticercosis is one of the leading preventable causes of epilepsy worldwide, estimated to contribute to up to 30% of epilepsy cases in areas where the disease is endemic. The total number of people with symptomatic or asymptomatic neurocysticercosis is estimated to be 2.56–8.30 million, according to the available data on the prevalence of epilepsy. These numbers may, however, be underestimates because of poor access of the groups at highest risk to diagnostic tests. Given this wide range, better understanding of the disease and its control is crucial, as is recognition of the lack of accurate information and the importance of more data on neurocysticercosis epidemiology. The internationally recognized criteria for diagnosis of neurocysticercosis include a requirement for neuroimaging techniques, such as computerized tomography (CT) and/or magnetic resonance imaging (MRI), ideally supported by serology. These facilities are not available in all settings, especially in rural areas of low-income countries, making it difficult to identify and treat patients. Additionally, there is controversy about the role, type and duration of anthelmintic, antiinflammatory and antiepileptic drug (AED) treatments for different forms of neurocysticercosis. These guidelines were developed to assist health-care providers in appropriate, evidence-based management of parenchymal neurocysticercosis. The guidelines do not address other forms of neurocysticercosis and do not include management of extraparenchymal disease (including cysticerci in the cerebral ventricles or subarachnoid space). The aim of the guidance is to improve decision-making to ensure appropriate patient care and to avoid misdiagnoses and inappropriate treatment of patients with neurocysticercosis. The guidelines were developed in a collaboration between the WHO departments of Control of Neglected Tropical Diseases and Mental Health and Substance Use.