Late presentation of HIV is common and is associated with several adverse outcomes including an increased risk of clinical progression, blunted immune recovery on highly active antiretroviral therapy and a greater risk of drug toxicity. Late presenters may have higher rates of poor adherence, exacerbated by the same factors that contribute to their late diagnosis, such as lack of knowledge about HIV and the benefits of highly active antiretroviral therapy. We review the definitions of, risk factors for and subsequent impact of late presentation. Evidence regarding how and when to start antiretroviral therapy, and with which agents, will be discussed, as well as issues surrounding vaccination and opportunistic infection prophylaxis for individuals with a low CD4 count. Finally, strategies to increase HIV testing uptake to reduce late presentation will be summarized.