A 7-year-old, otherwise healthy Peruvian boy presented with a 3-month history of an indurated centrofacial plaque. Histologic examination revealed a granuloma containing free-living amebae tentatively identified as Balamuthia mandrillaris. The patient failed to respond to tentative treatment. He was admitted to the intensive care unit 7 months later with neurologic manifestations of granulomatous amebic encephalitis, which proved fatal. The difficulty in diagnosing this rare presentation of cutaneous amebiasis, the challenge of treating the condition, and the morbidity and high mortality associated with cerebral involvement are discussed.