Neurocysticercosis is thought to be the most common helminthic infection of the central nervous system and its epidemiology is changing due to increasing travel and migration. Evidence to guide management of the intraventricular form is limited. We aimed to review the clinical presentation, diagnosis and treatment of intraventricular neurocysticercosis with reference to two recent cases seen at our institution. The intraventricular variant of neurocysticercosis is less common than parenchymal disease and usually presents with acutely raised intracranial pressure and untreated it progresses rapidly with high mortality. The diagnosis is based on imaging and serological tests but more invasive testing including histopathological examination of surgically acquired tissue specimens is sometimes required. Treatment is mainly surgical, using a neuroendoscopic approach if possible. Patients should also receive antihelmintic treatment with concomitant corticosteroids to reduce the incidence of shunt failure if a ventricular shunt is inserted and to treat viable lesions elsewhere.