West Nile virus, a flavivirus identified in Africa in the 1930s, appeared in the Western Hemisphere in 1999. Since its appearance, West Nile virus has caused nearly 40,000 cases of human disease in the US and more than 1,500 deaths, mostly among elderly persons with neuroinvasive disease. This review summarizes recent information regarding the clinical manifestations and prevention of West Nile virus infections in children, and emphasizes that although West Nile virus fever and neuroinvasive disease primarily affect adults, infants and children remain at risk of serious complications, including death, from this disorder.