Renal disease accounts for significant morbidity and mortality in patients with HIV-1 infection. HIV-associated nephropathy (HIVAN) is an important cause of end stage renal disease in this population. Although multiple genetic, clinical, and laboratory characteristics such as Apolipoproetin-1 genetic polymorphism, high viral load, low CD-4 count, nephrotic range proteinuria, and increased renal echogenicity on ultrasound are predictive of HIVAN, kidney biopsy remains the gold standard to make the definitive diagnosis. Current treatment options for HIVAN include initiation of combined active antiretroviral therapy, blockade of the renin-angiotensin system, and steroids. In patients with progression of HIVAN, renal transplant should be pursued as long as their systemic HIV infection is controlled.