One week after swimming in a man-made lake, a 9-year-old boy presented to the emergency department with headache, vomiting and lethargy. He had neck pain upon flexion and was unable to stand or walk. Cerebrospinal fluid examination revealed trophozoite and diflagellate forms consistent with Naegleria fowleri, an ameba species known to cause primary amebic meningoencephalitis. Despite aggressive management with amphotericin B and rifampin, he died 2 days later. This case report describes the clinical presentation, diagnostic findings and management of this uncommon but lethal entity.