Neurocysticercosis is an important cause of seizures worldwide and is endemic in most of Latin America, Sub-Saharan Africa, Southeast Asia, India, and China. Neurocysticercosis has profoundly different disease manifestations varying from asymptomatic presentation to life-threatening hydrocephalus. Clinical manifestations, pathogenesis, diagnostic methods, and optimal treatment vary with the location, number of lesions, and host response. Diagnosis is based on a combination of clinical presentation, neuroimaging findings, history of exposure, and serologic testing. Initial therapy should be focused on symptom management including seizure control and management of increased intracranial pressure. Emerging data are demonstrating that the optimal management approach varies with stage. Single enhancing or cystic lesions should be treated with albendazole and steroids. Patients with more than two cystic lesions should be treated with combination therapy with albendazole and praziquantel, whereas patients with hydrocephalus benefit from surgical management, especially with minimally invasive approaches.