Since the description of a new renal syndrome in patients with the acquired immunodeficiency syndrome (AIDS) in the middle 1980s, much has been learned regarding the association of human immunodeficiency virus (HIV) infection and renal disease. The HIV-associated renal diseases represent a spectrum of clinical and histopathologic conditions. In this review, epidemiologic and clinical aspects of HIV-associated renal diseases are presented. Particular attention is placed on the pathologic and pathophysiologic mechanisms involved in HIV-associated focal glomerulosclerosis, immune complex-mediated disease, and thrombotic microangiopathies. Pharmaceutical treatment options, including the use of glucocorticoids, angiotensin-converting enzyme (ACE) inhibitors, and highly active antiretroviral therapy, are discussed. The therapeutic option of renal transplantation is presented, with insight into new clinical and basic research supporting a possible role of immunosuppressive therapy in this already immunocompromised patient population.