Before the era of highly active antiretroviral therapy, more than 40% of human immunodeficiency virus (HIV)-infected children experienced renal complications. In sub-Saharan Africa, approximately 2.1 million children are infected with HIV-1. In the absence of antiretroviral therapy, young African children frequently died of AIDS-related complications before renal diseases could be manifested or diagnosed. As antiretroviral therapy has become more available, and their survival has increased, our experience in treating kidney disease in HIV-infected children has improved. This article discusses relevant clinical and pathologic findings related to kidney disease in HIV-infected children.