Myocarditis and myocardial infection with HIV-1 are the best-studied causes of cardiomyopathy in HIV disease. The introduction of highly active antiretroviral therapy has significantly reduced the incidence of myocarditis in HIV-infected patients living in developed countries. In developing countries, where the availability of highly active antiretroviral therapy is limited and the pathogenetic role of nutritional factors is greater, the incidence of HIV-associated myocarditis and cardiomyopathy is increasing with a high mortality rate for congestive heart failure. Immunomodulatory therapy may be helpful in adults and children with HIV-associated myocarditis and declining left ventricular function. Further controlled studies of this possible therapy are needed.