We report on two children with acquired immunodeficiency syndrome (AIDS), a 5-year-old girl and a 9-year-old boy, who presented with oral hairy leukoplakia (OHL) as a late clinical manifestation of perinatally acquired human immunodeficiency virus type 1 (HIV-1) infection. The reasons why OHL is a rare symptom in HIV-1-infected children, compared with HIV-1-infected adults, are discussed.