The long term clinical outcome for infants and children with the pediatric acquired immunodeficiency syndrome-related complex is unknown. This report describes our experience with 14 patients with acquired immunodeficiency syndrome-related complex who have been followed for 11 to 71 months since the onset of their symptoms. The most frequent clinical features at presentation were persistent generalized lymphadenopathy (14 of 14), hepatosplenomegaly (11 of 14) and a history of recurrent otitis media (7 of 14). Except for hypergammaglobulinemia (14 of 14) and reversed T4/T8 ratios (9 of 14), immunologic analyses, including in vitro responses to mitogens and antibody responses following immunization, revealed no consistent abnormalities. Over the course of follow-up, none of the patients have developed serious or opportunistic infections and 12 of 14 have shown catch up or age-appropriate growth. The T4/T8 ratios have remained stable in 8 of 11 and improved in 2 of 11 patients. Gradual regression of hepatosplenomegaly and lymphadenopathy has been noted patients. Although follow-up studies over a longer period are needed to confirm our observations to date, acquired immunodeficiency syndrome-related complex may represent a prolonged plateau in the course of human immunodeficiency virus infection in many infected children. Detailed immunologic evaluation of these patients may help to identify a subset of children that could benefit from periodic gamma-globulin or chronic antibiotic therapy.