Sixteen human immunodeficiency virus type 1 (HIV-1)-seropositive children aged 5 to 12 years (nine girls and seven boys), born to HIV-1-infected mothers, were diagnosed between 1984 and 1987 in Kigali, Rwanda. They were compared with a group of age- and sex-matched HIV-1-seronegative children consecutively selected from the outpatient department. Two subjects were asymptomatic. Chronic cough was the most frequent symptom (seven of 16 patients). The most common signs were short stature (12 of 16 patients), low weight for age (seven of 16 patients), chronic parotitis (eight of 16 patients), persistent generalized lymphadenopathy (seven of 16 patients), and pulmonary tuberculosis (four of 16 patients). Lymphoid interstitial pneumonitis was diagnosed on radiologic grounds in five of 16 patients. Evidence of perivasculitis in the fundus was noted in three of 16 patients. Two children died during the study period (mean duration of follow-up, 40 months; range, 27 to 62 months); none of the other children had life-threatening infection or loss of developmental milestones. Immunologic assessment in the 16 children revealed high levels of IgG, decreased CD4+/CD8+ ratio, and skin test anergy. Endocrinologic investigations revealed normal thyroid function and normal basal human growth hormone levels but low basal insulinlike growth factor I levels (0.21 +/- 0.07 vs 0.44 +/- 0.20 U/mL for controls). In Kigali, perinatally HIV-1-infected children surviving beyond 5 years of age often present with moderate signs and symptoms, principally pulmonary involvement, chronic parotitis, and persistent generalized lymphadenopathy. Short stature is the major clinical manifestation in these patients and may be due, in part, to low growth hormone secretion rather than to malnutrition.