ABSTRACT. Objectives. To compare language development in infants and young children with human immunodeficiency virus (HIV) infection to language development in children who had been exposed to HIV but were uninfected, and (among subjects with HIV infection) to compare language development with cognitive and neurologic status.Design. Prospective evaluation of language development in infected and in exposed but uninfected infants and young children.Setting. Pediatric Infectious Disease Clinic, State University of New York-Health Science Center at Syracuse.Subjects. Nine infants and young children infected with HIV and 69 seropositive but uninfected infants and children, age 6 weeks to 45 months.Results. Mean Early Language Milestone Scale, 2nd edition (ELM-2) Global Language scores were significantly lower for subjects with HIV infection, compared with uninfected subjects (89.3 vs 96.2, Mann-Whitney U test). The proportion of subjects scoring >2 SD below the mean on the ELM-2 on at least one occasion also was significantly greater for subjects with HIV infection, compared with uninfected subjects (4 of 9 infected subjects, but only 5 of 69 uninfected subjects; Fisher's exact test). Seven of the 9 subjects with HIV infection manifested deterioration of language function. Four manifested unremitting deterioration; only 1 of these 4 demonstrated unequivocal abnormality on neurologic examination. Three subjects with HIV infection and language deterioration showed improvement in language almost immediately after the initiation of antiretroviral drug treatment. Magnetic resonance imaging or computed tomography of the brain were performed in 6 of 7 infected subjects with language deterioration, and findings were normal in all 6. ELM-2 Global Language scaled scores showed good agreement with the Bayley Mental Developmental Index or the McCarthy Global Cognitive Index (r ؍ 0.70). Language deterioration, or improvement in language after initiation of drug therapy, coincided with or preceded changes in global cognitive function, at times by intervals of up to 12 months.Conclusions. Language deterioration occurs commonly in infants and young children with HIV infection, is seen frequently in the absence of abnormalities on neurologic examination or central nervous system imag-